RHEUMATISM (Greek rheumatismos expiration, rheumatic suffering; synonym: acute rheumatic fever, Sokolsky's disease — Buyo, rheumatic fever, Buyo's disease, true rheumatism) — a general inflammatory disease of connecting it is woven with preferential localization of process in cardiovascular system, developing at the persons predisposed to it, mainly young age (7 — 15 years), in connection with an infection a beta and hemolitic streptococcus of group A.
- 1 HISTORY
- 2 STATISTICS
- 3 The AETIOLOGY AND the PATHOGENY
- 4 The PATHOLOGICAL ANATOMY
- 5 CLASSIFICATION
- 6 The MAIN CLINICAL DISPLAYS of RHEUMATISM, ITS CURRENT AND DIAGNOSIS
- 6.1 A rheumatic carditis (rheumatic carditis)
- 6.2 Rheumatic polyarthritis
- 6.3 Defeat of a nervous system
- 6.4 Mental disorders
- 6.5 Damages of lungs
- 6.6 Damage of abdominal organs
- 6.7 Damage of kidneys
- 6.8 Damage of skin and hypodermic cellulose
- 7 CRITERIA of DIAGNOSIS of RHEUMATISM
- 8 TREATMENT
- 9 PREVENTION
- 10 Tables
the First data on R. as about a disease of joints meet in Ancient Chinese medicine. Hippocrates (460 — 377 BC) the clinic of the classical acute rheumatic migrating polyarthritis is described and the fact of preferential development of a disease in persons of young age is noticed. K. Galen was, obviously, the first who according to the views of gumoralist dominating into that pore placed a concept rheumatism. R.'s allocation as general disease of an organism, recognition of the rheumatic nature of a chorea happened in 17 century and is connected with works of G. Bail-lou and T. Sydenham. The first researchers who recognized communication of rheumatism with damage of heart were probably the English doctor Pitkern (D. Pitcairn, 1788) and the Russian doctor F. Volkovinsky (1818). However before the emergence of works of Zh. Buyo (1835, 1840) and G. I. Sokolsky (1836, 1838) who gave independently from each other the accurate description of rheumatic damage of heart with formation of defects of its valves among doctors idea of R. only as about a disease of joints dominated. The further stage of development of the doctrine about R. should be considered recognition it a number of researchers (S. P. Botkin, 1888; G. M. Mal-kov, 1900, etc.) general organ disease with involvement in pathological process not only hearts and joints, but also other bodies. Since works of L. Ashoff who described granulomas, characteristic of R., in a myocardium, L. Ashoff, V. T. Talalayev, M. A. Skvortsov, I. V. Davydovsky, N. A. Krayev-sky, A. I. Strukov, G. V. Oryol, H. N. Gritsman, etc. was carried out a cycle of the anatomic and gisto-morphological researches which led to recognition of almost obligatory damage of heart at R. and to interpretation of a disease from klinikoanatomichesky positions. Inclusion of rheumatism in group of allergic, and then infectious and allergic diseases was based on G. M. Malkov (1900) works, Vayntrauda (W. Weintraud, 1913), Klinge (F. Klinge, 1932), D.E. Aljperna (1934), N. D. Strazhesko (1935), Koberna (A. F. Coburn, 1932), V. I. Ioffe (1962), I. M. Lyampert et al. (1963), clinical and theoretical generalizations of M. P. Konchalovsky, M. V. Chernorutsky, etc. The original domestic direction in understanding of mechanisms of development of rheumatic process were the attempts of justification of a neurogenic and infectious and neurogenic pathogeny of R. presented in A. D. Speransky (1934) works, N. I. Leporsky (1933), A. I. Nesterova (1952), I. V. Vorobyov (1952), V. G. Vogralik (1953), M. V. Chernorutsky (1955), etc. They determined the facts confirming participation of a nervous system in formation of many clinical manifestations to R. Odnako a role of a nervous system was revaluated. At the same time the saved-up actual material, undoubtedly, can be useful during the development of the system principle in formation of ideas of a pathogeny of a disease. One of the leading places in development of modern ideas of clinic of R. belongs to domestic clinical physicians. In addition to G. I works. Sokolsky and S. P. Botkin, an essential contribution to studying of clinical forms, options of a current, diagnostic criteria of R. were the works S. S. Zimnitsky, N. D. Strazhesko, M. P. Konchalovsky, G.F. Lang, A. I. Nesterov, B. A. Egorov, M. V. Chernorutsky, V. E. Nezlin, E. M. Gel-stein, P.E. Lukomsky, A. B. Volovik, O. D. Sokolova-Ponomareva, A. V. Dolgopolova and many other domestic therapists and pediatricians devoted to these problems. It is especially necessary to note A. A. Kisel's merits, to-ry long before emergence of work of Jones (T. D. Jones) allocated five «absolute symptoms of rheumatism»: rheumatic small knots, a ring erythema, a chorea, the migrating polyarthritis and a carditis. Therefore to call naturally diagnostic criteria of rheumatism criteria of Kissel — Jones.
The last period in development of the doctrine about R. is characterized by recognition of an etiological role [3-gemoli-ticheskogo streptococci of group A and promotion by A. I. Nesterov (1973) kliniko-immunological concepts of a pathogeny of R. what the main achievements in fight against R. which led to considerable decrease in incidence in our country are connected with.
R. is widespread around the world. R.'s incidence significantly does not depend on klimatogeografichesky conditions, but is accurately connected with economic level of development of this or that country (considerable prevalence of a disease in developing countries and rather small — in economically developed). However the existing information on incidence and prevalence of R. is not always authentic that considerably is explained by absence in the majority of the countries of obligatory registration for the first time of the diseased, difficulty of diagnosis and quite often inadequate population epidemiological researches. Data on mortality from rheumatism are more authentic. E.g., in Denmark, according to Stollerman (G. N of Stollerman, 1975), mortality from R. in 100 years (1862 — 1962) decreased with 200 to And on 100 000 population. In the Soviet Union essential decline in mortality and primary incidence is noted. So, in the ten years' prospective studying of primary incidence of R. which is carried out by Institute of rheumatology of the USSR Academy of Medical Sciences (at the semi-million population of two districts of Moscow) its decrease from 1970 to 1979 by 2,5 times (from 0,17 to 0,07 on 1000 people), and among children, according to 3 is shown. P. Annenkova (1981), even three times — with 0,54 to 0,18 on 1000 children's population. Similar data are provided also by other researchers. This favorable dynamics is connected with such social factors as growth of welfare of the population of our country, improvement of living conditions, and also considerably and results of evidence-based system of the organization of the rheumatological help and complex prevention of a disease. At the same time progressive decrease in incidence in the 60th years both in the USSR, and in other economically developed countries was replaced by a tendency to stabilization of indicators in the last decade.
At the same time prevalence of rheumatic heart diseases still remains high (1,4% of all population, according to epidemiological these L. I. Benevolenskoy et al., 1981), however this circumstance is connected with a number of positive tendencies in the state of health of this category of the population: increase in life expectancy of patients and decline in mortality due to the lack of R.'s activity thanks to carrying out a universal bitsillinoprofilaktika and timely antirheumatic treatment.
The AETIOLOGY AND the PATHOGENY
the Leading concept of development of R. is the streptococcal theory confirmed not only theoretical researches, but also more than twenty-year-old. treatment-and-prophylactic activity of rheumatologists of the whole world.
Usually precedes R.'s development quinsy (see) or an acute respiratory disease, groups A called by a r-haemo-lytic streptococcus (see. Streptococci ), to what detection at the prevailing sick R.' most in blood serum of high credits of various antistreptococcal antibodies indirectly testifies: antistreptolysin-0 (ASL-O), an antistreptogialuro-nidaza (ASG), anti-Streptokinasa (ASK), an anti-deoxyribonuclease In (Anti-DNA-ases B). Value of a streptococcal infection in R.'s development is confirmed also by the fact that the disease can be prevented by careful treatment of quinsy and inflammatory diseases of a nasopharynx. R.'s communication with a streptococcal infection is proved also by the data of epidemiological researches which are carried out at boarding schools, military colleges, barracks for the military personnel. Correlations between incidence inf are established. diseases of a nasopharynx and acute rheumatic attack, coincidence of curves of incidence of quinsy, scarlet fever and River. Epidemiological researches also show that one of conditions of emergence of primary R. is weight of a streptococcal infection and a long carriage of a streptococcus at insufficient treatment of quinsy. According to Baum (J. Baum, 1979), credits of antistreptococcal antibodies are higher at those patients, at to-rykh R. developed after quinsy.
A. S. Labinsk (1975) established existence in a pharynx of a streptococcus in the first 2 weeks of a disease at 100% of patients primary and at 90% of patients with returnable rheumatism. According to E. P. Ponomareva (1971), release from a nosytelstvo of a streptococcus happens quicker at primary, than at returnable R., but in both cases long administration of penicillin and Bicillinum is necessary.
So far the question of existence of «revmatogenny» strains of a streptococcus is discussed. According to Stollerman (G. The N of Stollerman, 1975), treat «revmatogenny» strains of the streptococci deprived of a lipoproteinaza («a factor of opacification»). He assumes that development of a carditis, polyarthritis, a chorea and other clinical manifestations of R. is caused different «roar-matogennymi» by strains of a streptococcus. This point of view is contradicted by E. P. Ponomareva's data (1971) and A. S. Labinsk et al. (1972), shown that in sick R.' pharynx in an active phase of a disease 15 — 20 serotii of a streptococcus are found, to-rye can be also at Clinically healthy faces and patients with other streptococcal infections. However the problem of «revmato-gene» strains of a streptococcus still draws attention in connection with the known fact of development of epidemic quinsies, later to-rykh incidence of rheumatism is 10 times higher, than after sporadic. According to A. S. Labinsk and V. D. Belyakova (1978), formation of «the revma-togenny potential» of a streptococcus happens during epidemic process, in the so-called closed collectives. R.'s development after the postponed streptococcal infection is rather connected, however, not with a special strain of a microbe from «revmatogenny-ma» properties, and with massiveness of an infection and duration carry-gelstva a streptococcus — important factors in development of a protivostrep-gokokkovy immune response, without to-rogo, as well as without the infection, R.
Svoyeobrazna a role of a streptococcal infection in development of returnable rheumatism cannot develop. In this case, according to Stollerman (1964), longer carriage of a streptococcus in a nasopharynx and big sensitivity of patients to infection with a serotype of a streptococcus, new to them, however without development of an adequate anti-streptococcal immune response (credits of antistreptococcal antibodies are usually low) is observed, as formed the basis for searches of other etiological factors, napr, viruses. G. D. Zalessky and it sotr. attach significance in R.'s development, especially returnable, developing against the background of heart diseases, to Koksaka A13 viruses (a so-called virus of rheumatism), and V.P. Kaznacheev — virus and streptococcal association.
Recently the question of a role of casing-free forms of a streptococcus (see L-forms of bacteria) in R.'s development, mainly returnable is actively developed. Detection of antibodies to L-forms of a streptococcus preferential at patients with a long, continuous and recurrent current of R. gives the grounds to think of a role of these transformed streptococci in progressing of River. According to V. D. Timakov and G. Ya. Kagan (1973), A. S. Labinsk and V. D. Belyakova (1978), etc., formation of L-forms of a streptococcus leads to long preservation it in an organism of the patient, and their reversion (i.e. reverse transformation) in an initial look promotes R.'s recurrence, to-rye can develop without infection with a new serotype of a streptococcus, as a result of activation of a persistent streptococcal infection in an organism.
Primary R.'s development after the postponed streptococcal infections only in a part of persons testifies to value of predisposition to development of a disease; according to WHO data (1968) — from 0,1 — 0,3% in the general population and at 3% in nek-ry military camps and barracks. Really, many data on continuous communication of two factors at R. are saved up — a streptococcal infection and predisposition. So, it is known that in sick R.' families more high frequency of a carriage of a streptococcal infection and R.'s prevalence, than in population is noted. Not only intensity of a streptococcal infection, but also feature of reaction of an organism to streptococcal antigens, in particular duration of an anti-streptococcal hyper immune response are important for R.'s development that is especially obvious at primary rheumatism (antistreptococcal antibodies remain at these patients in high credits and after approach of clinical laboratory remission).
The reasons of unusual duration of the anti-streptococcal hyperimmune answer at sick R. are not clear yet. The role of genetically caused defect of elimination of a streptococcus from an organism, the raised susceptibility to an intrahospital streptococcal infection is discussed, edges develops at sick R. after the first attack.
Predisposition to R. is not limited only to special reactivity of immune system on a streptococcal infection, and, on - visible mu, has multifactorial character. Check of various genetic hypotheses showed that the mode of inheritance of R. corresponds to multifakto-rialny models. Inspection of families of sick R. revealed existence of a disease at 9,6% of their parents and at 8,2% of children that by 4 times exceeds R.'s frequency in population. Also more frequent development of R. in sibs (brothers and sisters of the patient) is established if any of their parents is sick R. Rol of genetic predisposition at R. confirms and higher spread of a disease among monozygotic twins, than dizygotic is essential (respectively 37,7% and 6,6%).
Researches of genetic markers showed that among sick R. persons with blood groups are more often observed And yes V. U of sick R. with a blood group In were found higher credits Antistreptoli-zina-oh, that can testify to hypersensitivity to a streptococcus of people of g this blood group.
In recent years intensive researches of communication of individual rheumatic diseases with immunogenetic factors, in particular by system of histocompatibility antigens — HLA are conducted (see. Immunity transplant, histocompatibility antigens ). Zabriskie (J. Century of Zabriskie, 1976) paid attention to decrease in frequency of HLA AZ at sick R. of children, and Joshinoya et al. (1980) revealed at them the increased frequency of HLA B5. Baum (J. Baum, 1979) reports about two series of researches: in one authentically increased frequency of HLA B35 was found in sick R., and in the presence of a rheumatic carditis HLA B18 antigen was registered more often; in other series — distinctions at sick R. in comparison with population it was revealed not.
The streptococcus makes on a human body impact various intracellular antigens (see) and extracellular exoenzymes. Among the first the M-protein of a cell wall (a factor of virulence), hyaluronic acid of the capsule and numerous antigens of a cytoplasmic membrane are essential. Various waste products of a streptococcus — exoenzymes, such as streptolysin-0 and S, hyaluronidase, Streptokinasa, a deoxyribonuclease In, etc. concern to the second. Both those, and others cause development of an immune response with the increased formation and long circulation of various antistreptococcal antibodies (see), break phagocytal activity of neutrophils that promotes a long persistention of a streptococcus.
Among mechanisms there is development of such clinical manifestations of R. as a carditis, polyarthritis, a chorea, a ring-shaped erythema, the greatest significance is attached to immune type of an inflammation, immunopathological reactions, to-rykh streptococcal antigens and antibodies take the activest part in development (see. Immunopathology ). It is supposed that in development of fabric damages at R. cross reacting antibodies, i.e. such antibodies can be of great importance, to-rye, being formed in response to antigens of a streptococcus, react with fabric antigens of an organism. Cross-reaction between polysaccharide of a streptococcus of group A and epithelial klet-us was found (epitelioretikulotsita) of a thymus with what, in opinion I. M. Lyampert et al. (1975), disturbances of immunosuppressor function of a thymus and functioning of T lymphocytes, development of cell-mediated autoimmune reaction are connected.
Also other cross reacting antibodies, in particular the streptococcal antibodies which are cross reacting with sarkolemmalny antigens of a myocardium are found.
Pathogenetic value of the circulating anti-cardial antibodies certainly is confirmed by detection in tissue of a myocardium of deposits (deposits) immunoglobulins (see) and complement (see) what Kaplan (M. N. of Kaplan) with sotr for the first time paid attention to. (1964) at a research of heart of the child who died at the phenomena of a pancarditis. The fixed immunoglobulins are found in a myocardium died from R. or in fabric of ears of the auricles removed at a commissurotomy. At sick R. come to light as well other antibodies, to-rye can have pathogenetic value. In this regard the circulating antibodies to components of connecting fabric — to fibroblasts, a structural glycoprotein, proteoglycans are of the greatest interest (see. Autoantibodies ).
Also disturbances of a cell-mediated immune response, napr, to cytoplasmatic and membrane antigens of a streptococcus, to streptococcal membranes and cross reacting antigens are noted. T. D. Bulanova (1976) and Williams's works (M. N. of Williams) et al. (1976) showed the disturbance in the joint answer from cellular and humoral links of immunity (see) which is shown in change of a quantitative ratio of T - and V-lymphocytes (see. Immunekompetentny cells ) and disturbance of their functioning.
At R. find the circulating cell-bound immune complexes. V. A. Nasonova with sotr. (1982) showed the high frequency of the circulating cell-bound immune complexes at R., in structure to-rykh are defined anti-streptolysin - 0 and S4 component of a complement. More than at sick R.' half, at to-rykh cell-bound immune complexes came to light, various types of disturbances of a cordial rhythm and conductivity are noted, and 1/3 patients have an increase in internal dimensions of a left ventricle of heart and decrease in sokratitelny ability of a myocardium (according to an echocardiography). Correlation between the high content of the circulating cell-bound immune complexes and development of a chorea in children, sick R., was established by Williams with sotr. (1979). It is possible to think that the streptokkovy infection promotes activation of various mediators of an inflammation and development of microcirculator disturbances.
Thus, R. is closely connected with a streptococcal infection (| a 5-hemolitic streptococcus of group A), about a cut it not only begins, but edges further is the reason of exacerbations of a disease. It is not excluded that at a number of patients with a chronic current of R. (long, latent, continuous retsidi-viruyushchim) a streptococcal infection in the form of casing-free L-forms is long persistirut in bodies and fabrics, promoting preservation of pathological process, and the kardiotropnost of many antigens and enzymes of a streptococcus found in an experiment (the shell, membrane, cytoplasmatic) — to development and progressing of a rheumatic carditis. An important role is played at the same time by an unusual immune response in the form of excess humoral reaction to exoenzymes and antigens of a streptococcus, including its L-forms, ability of antistreptococcal antibodies to react cross with fabric antigens and to form the circulating cell-bound immune complexes that leads to development of immunopathological processes. Apparently, also disturbance of a cellular link of immunity in the form of accumulation of a clone of lymphoid cells, sensibilized to the streptococcal and cross reacting antigens matters. All this promotes formation of a rheumatic inflammation, both nonspecific exudative, and specific granulematozny (an ashoff-talalayevsky granuloma).
The PATHOLOGICAL ANATOMY
is the cornerstone of fabric changes at R. system disorganization of connecting fabric (the deepest and progressing in heart and vessels) in a combination to specific proliferative and nonspecific exudative and proliferative reactions, and also defeat of vessels of a microcirculator bed.
Since 50th years evolution of kliniko-morphological manifestations R.'s (pathomorphism) is noted that it is connected first of all with actively carried out prevention and treatment of a disease. First of all it belongs to the acute nonspecific exudative and infiltrative inflammatory processes connected with giperergichesky reactions. In particular, the acute synovitis, a polyserositis, rheumatic small knots and an encephalomeningitis meet much less often. By data A. I. Lukina (1971), the pathomorphism of a rheumatic carditis consists in sharp reduction of cases of exudative myocarditis and a pericardis, multivalve heart diseases, a rarity of detection of ashoff-talalayevsky granulomas. At the same time the isolated defects of the mitral valve became frequent.
V. T. Talalayev (1929) for the first time allocated in a cycle of rheumatic process exudative alterativnuyu, granulematozny and sclerous stages. Later A. I. Strukov (1961, 1963) on the basis of the histochemical analysis showed that the first stage comes down not only to to an inflammation (see), and it is shown by a peculiar disorganization (dystrophy) of connecting fabric. He suggested to divide it into stages of mucoid swelling and fibrinoid changes.
Mucoid swelling (see. Proteinaceous dystrophy ) — the earliest, superficial reversible phase of disorganization of connecting fabric which is characterized by mucoid (basphilic) hypostasis, diffuse and focal metachromasia (see), coming to light during the coloring by toluidine blue. At the same time there is not only a true accumulation of acid glikozaminoglikan due to their synthesis by cells, but also «demaskirovka» of reactive groups of glikozaminoglikan owing to dissociation of proteinaceous and carbohydrate complexes of connecting fabric under the influence of the activated proteolytic enzymes. Accumulation of free glikozaminoglikan, especially hyaluronic acid (see), causes the increased hydration of the main substance of connecting fabric. Collagenic fibers bulk up, there is their razvolokneniye, but colourful properties do not change. Fibers become unstable to action of a microbic collagenase that is connected with change of a cement substance of fibers (proteoglycans). At a submicroscopy the razvolok-neniye of fibers on fibrilla comes to light, splitting of fibrilla on subfibrilla (fig. 1), strengthening of coloring ruthenic red, contacting carbohydrate components is more rare.
Fibrinoid changes (swelling, a necrosis) represent the following, irreversible phase of disorganization of connecting fabric (fibrinoid). According to A. I. Strukov (1974), fibrinoid at R. is connected with immunocomplex damage of a vascular bed and connecting fabric. It is followed by receipt to sites of damage of proteins of plasma, generally globulins (see) and fibrinogen (see), to-rye form patol. complexes with the disorganized proteoglycans and the destroyed collagenic fibers. The centers of fibrinoid are characterized by consecutive swelling, homogenization, granular and glybchaty disintegration of collagenic fibers, easing of their fuchsinophilia and strengthening of a pikrinofiliya during the coloring according to Van-Gizona, argirofiliy, CHIC reaction, red coloring across Mallori or Geydengayna (the evidence of availability of plasma proteins). Allocation (1961) by A. I. Strukov on the basis of colourful reaction to fibrin of two forms of fibrinoid (with fibrin and without fibrin) is conditional since biochemical, immunohistochemical, polarizing and optical and electronic and microscopic data demonstrate that fibrin comes to light at all forms of fibrinoid. At a submicroscopy in fibrinoid all phases of destruction of collagenic fibrilla are found: adhesion, loss of a leriodichnost, fragmentation, splitting on sub - and protofibrils with a chaotic arrangement of the last. At a fibrinoid necrosis also destruction of cellular elements is found (see. Fibrinoid transformation ).
In the first two stages disorganization of connecting fabric is followed by its limfogistiotsitarny infiltration.
Granulematozny reaction (the third stage of rheumatic process) is shown in heart in the form of the specific rheumatic granuloma described for the first time by L. Ashoff (1904) and in more detail V. T. Talalayev (1929) and therefore the ashoff-talalayevsky granuloma, or a small knot carrying the name (see the Granuloma). The granuloma is characterized by accumulation of cells with hyperchromic kernels and basphilic cytoplasm around the small centers of a fibrinoid necrosis (an early stage, or a phase of formation of a granuloma). Then the granuloma accepts a characteristic rozetkovidny structure (fig. 2) with the advent of large one - or multinucleate cells (ashoffsky cells) performing resorptive function (mature, or «blossoming», a granuloma). Further cells of a granuloma are extended, gaining lines of fibroblasts, fibrinoid masses disappears, neogenic collagenic fibers (the «withering» and cicatrizing granulomas) appear. The cycle borrows, according to V. T. Talalayev, 3 — 4 months.
Cells of a mature granuloma are histochemical characterized by the high content of RNA, a glycogen, oxidation-reduction enzymes, esterases, acid phosphatases, aminopeptidases; they fix immunoglobulins and cell-bound immune complexes. At a submicroscopy in early and mature granulomas cells like macrophages — macrophagocytes (fig. 3) though which do not have the expressed ability to phagocytosis prevail. The resorption of fibrinoid is carried out by its intercellular lysis by sekretirovan-ny lizosomalny enzymes and the subsequent pinocytic (see).
In the sclerosed granulomas fibroblasts prevail. As transformation of macrophages in fibroblasts on modern representations is impossible, the last are probably a product of an independent line of a differentiation.
In granulomas, in addition to ashoffsky cells and fibroblasts, so-called cells of Anichkov (chromatin in their kernel is located in the form of «caterpillar»), lymphocytes, plasmatic and mast cells, single neutrophils (generally on the periphery of granulomas), and in a myocardium as well colossal cells of a myogenetic origin meet. Fassbender (N. of G. Fa-ssbender, 1975), in addition to a classical resorptive granuloma, describes also productive granuloma without the previous fibrinoid and a myogenetic granuloma. Typical ashoff-talalayevsky granulomas have nosological and organ specifics, arising only in heart (the endocardium covering walls of cardial cavities i.e. a pristenochny endocardium, valves, a myocardium, is more rare in an outside cover of vessels). In other fabrics, napr, circumarticular, intermuscular, peritonsillar, nonspecific granulemopodobny cellular reaction of a macrophagic origin is found.
Sclerosis (see) is a final stage of rheumatic process. It develops as an outcome of granulomas and nonspecific inflammatory processes (a secondary sclerosis) or as the result of fibrinoid changes without the subsequent formation of granulomas (primary sclerosis, a hyalinosis). The hyalinosis of fabric represents consolidation and homogenization of fibrinoid masses and their transformation into the fabric poor in cells similar to a hyaline cartilage (see the Hyalinosis). At a submicroscopy among granular material find chaotic collagenic fibrilla. Formation of the stable complexes consisting of plasma proteins, cell-bound immune complexes and the destroyed collagenic fibers is the cornerstone of a hyalinosis. In the hyalinized fabric salts of calcium (dystrophic calcification), most often in valves of heart drop out.
In addition to the above described process of disorganization of connecting fabric and a specific granulomatosis, in R.'s clinic not a smaller role is played by the nonspecific fabric reactions in essence defining activity of a disease. Treat them acute exudative in-filtrativnoye an inflammation of a myocardium, an endocardium, a pericardium (especially at children), joints and serous covers; focal (diffusion are more rare) perivascular limfogistiotsitar-ny infiltrates in connecting fabric of various bodies.
To number nonspecific, but extremely important for a pathogeny and R.'s morphogenesis of processes changes of vessels of microcompasses - an even bed belong. They are found in all bodies, is especially distinct at special techniques of impregnation of filmy drugs. These changes are most of all expressed in capillaries and arterioles and are shown in a focal loosening and consolidation of basal membranes, swelling, hypostasis, desquamation and the subsequent proliferation of an endothelium (a rheumatic endotheliosis) that is noted also at a submicroscopy. In arterioles and arteries the destructive and productive vasculitis, perivascular cellular infiltration, sometimes plasmatic treatment, fibrinoid swelling and a necrosis of a vascular wall develops, thrombosis of a vessel is more rare (see. Vasculitis ).
Rheumatic carditis. The Kliniko-morfologichesky picture P. and disease severity generally are defined by defeat of connecting tissue of heart and sokratitelny myocardium. Rheumatic process strikes all covers of heart, and depending on preferential localization allocate a rheumatic endocarditis, myocarditis and a pericardis. Simultaneous development of inflammatory process in all covers of heart carries the name of a pancarditis (see). At all variety of fabric changes in heart they keep within the following main manifestations: disorganization of connecting fabric of valves, pristenochny endocardium, epicardium, pericardium and stroma of a myocardium; specific granulematozny reactions in the form of ashoff-talalayevsky granulomas; the nonspecific exudative and proliferative reactions which are shown in an inflammation of an endocardium, a myocardium and a pericardium; defeat circulatory and limf, vessels of heart of all calibers — from large coronary arteries to a microcirculator bed; changes of a sokratitelny myocardium in the form of a hypertrophy, an atrophy, dystrophy, a necrobiosis and intracellular regeneration of heart muscle fibers; defeat of the carrying-out system and nervous elements of heart; sclerous processes, to-rye are the result of all these changes.
Particular interest at a rheumatic carditis raises a question of communication of disorganization of connecting fabric and a granulomatosis with activity of rheumatism. M. A. Skvortsov (1938) specified that the clinical symptom complex of the rheumatic attack is caused by exudative nonspecific manifestations, and specific morfol. changes correspond to the mezhpristupny period and characterize clinically imperceptible course of process.
A. I. Strukov on the basis of long-term observations put forward the provision on rheumatism as continuous process, to-ry proceeds in the form of alternation of four phases of disorganization, but without obligatory existence of granulomas. They on section material are found in recent years very seldom, according to Roberts and Virnani (W. Page of Roberts, Virnani, 1978), in only 2% of cases. At a biopsy of an ear of the left auricle taken in time of a mitral commissurotomy, granuloma according to Todesi and Wagner (To-deshi, Wagner, 1955), A. I. Struko-va, R. A. Simakova (1965), H. N. Gritsman (1971), Roberts and Virnani (1978), come to light in 20 — 80% of cases. At the same time lack of correlation between existence of granulomas and clinical laboratory indicators of activity of River is noted.
To clinical activity of process there correspond such nonspecific exudative and proliferative signs as swelling, proliferation and desquamation of an endothelium of an endocardium and vessels, hypostasis of an endocardium and stroma of a myocardium, reaction of connective tissue cells in the form of focal or diffusion limfogistiotsitarny infiltration and fibroblastichesky proliferation to a large extent. At a certain expressiveness of these changes there is clinically diagnosed rheumatic carditis.
Myocarditis (see) at R. subdivide into three main morfol. forms: nodular productive myocarditis with development of specific granulomas in a stroma of a myocardium, most often in perivascular spaces; diffusion interstitial exudative myocarditis with hypostasis and the expressed nonspecific neutrophylic and lymphocytic infiltration of a stroma of a myocardium; focal and interstitial myocarditis with dominance of limfogistiotsitarny elements in infiltrate. Fassben-der (1975), except productive and exudative forms of rheumatic myocarditis, allocates myshechnoagressivny, edges it is characterized by active cellular destruction of muscle fibers of a myocardium.
From all departments of heart inflammatory process the left ventricle and papillary muscles most often are surprised, then it is consecutive: interventricular partition, right auricle, right ventricle, left auricle. The diffusion interstitial exudative myocarditis affecting several departments of heart occurs seldom, generally at children at very active process, sometimes at elderly patients now. It is, apparently, manifestation of giperer-gichesky reaction and has clinically adverse current. M. A. Skvortsov (1938) who for the first time in detail described this form of myocarditis at children noted serous hypostasis, neutrophylic and limfogistiotsitarny infiltration (see tsvetn. fig. 4), to-rye can accompany specific granulematozny process, but can be and independent of it. Often at the same time there are centers of a necrosis of muscle fibers.
It is known, however, that dystrophy, the necrobiosis and even a necrosis of muscle fibers of a myocardium can develop also without myocarditis, being a consequence of changes of microcirculation, disturbances of metabolism or direct effect of streptococcal toxins and antibodies. Such changes, amplifying at each rheumatic attack, disturbances of sokratitelny function of a myocardium and a decompensation of blood circulation are the cornerstone. As ultrastructural expression of dystrophic and necrobiotic changes of muscle fibers serve hypostasis of a sarcoplasm of cardiomyocytes, swelling, consolidation, destruction and lysis of mitochondrions, dissociation and destruction of myofibrils, kontrakturny reduction and a partial lysis of sarcomeres, vacuolation and a lysis of tubular elements of a sarcoplasmic reticulum, increase in number of lysosomes and granules of a pigment, accumulation of fatty vacuoles, polymorphism, pycnosis and fragmentation of kernels. The ultrastructural picture differs in mosaicity that is expressed in alternation of the changed and not changed cardiomyocytes, and also cells with dominance of signs of intracellular regeneration. Histochemical in a myocardium it is observed fatty dystrophy (see) and proteinaceous dystrophy (see), decrease in maintenance of a glycogen, change of activity of enzymes, fuchsinophilia.
In the carrying-out system of heart at R. fatty dystrophy, a fuchsinophilia, an atrophy and fragmentation of the carrying-out muscle fibers, and also the phenomena of fibrosis are also found. In nervous cells intramural gangliyev hearts are observed vacuolation of cytoplasm, pycnosis and a chromatolysis of kernels, hypostasis and fragmentation of axons and a razvoloknenpa of neurofibrilla in nerve fibrils.
Pericardis (see) at R. has character serous, serofibrinous or fibrinous; at the last formation of commissures is quite often observed. Are quite often combined with a pericardis serous or serofibrinous pleurisy (see) and peritonitis (see) that allows to speak about the rheumatic polyserositis which is usually arising in the acute period of a disease (see. Polyserositis ).
Changes of vessels of heart play an important role in a pathogeny of a rheumatic carditis. In vessels of microcompasses - an even bed of heart (capillaries, arterioles, venules) there is a swelling of basal membranes, hypostasis, desquamation and proliferation of an endothelium. In larger vessels find plasmatic treatment, fibrinoid swelling and a segmented necrosis of walls, a productive vasculitis, sometimes a thrombovasculitis, perivascular infiltration, later — a sclerosis and a hyalinosis. On R.'s ratio and atherosclerosis a consensus is absent, but G. L. Dearman and V. D. Sadchikov (1975) who found activation of a lipase in vessels at R. consider, as well as L. E. Kremenetskaya (1970) that at rheumatic defects the stenosing coronary atherosclerosis arises less often.
Vessels of heart are also exposed to changes limf. At acute exudative myocarditis or a pericardis are noted sharp expansion limf, capillaries and lacunas, dissociation of an endothelium, lymphostasis (see) and limforragiya (see the Lymphorrhea). At a latent current of a rheumatic carditis against the background of reorganization and partial reduction limf, networks with fibrosis of walls of vessels are observed sites dilatatsin a gleam, a new growth of capillaries, limfogis-thiocytic infiltration. Rezorb-tsionny insufficiency of a lymphokinesis leads to strengthening of dystrophy of a myocardium and a sclerosis on the course limf, vessels.
The result of all above described processes at a rheumatic carditis is pass (see), to-ry, thus, it is possible to consider a consequence of disorganization of connecting fabric (primary sclerosis and a hyalinosis), a specific granulomatosis (a secondary sclerosis), nonspecific inflammatory reactions, metabolic necroses of muscles (melkoochagovy fibrosis), disturbances haemo - and limfotsirkulyation.
At a rheumatic endocarditis shutters of valves of heart (a valve endocarditis, or a valvulitis), tendinous chords of heart (a chordal endocarditis) and a pristenochny endocardium (a parietal endocarditis) are surprised. Most often valve develops endocarditis (see). Frequency of defeat of valves of heart has such sequence: mitral (the left atrioventricular), valves of an aorta, three-leaved (right atrioventricular) valve and exclusively seldom valves of a pulmonary trunk.
A. I. Abrikosov (1947) allocates four forms of a rheumatic endocarditis: diffusion, acute warty, fibroplastic, returnable and warty. The diffusion (idle time) endocarditis (or a valvulitis, according to V. T. Talalayev) is characterized by mucoid and fibrinoid swelling superficial (is more rare deep) layers of shutters of valves of heart with moderate histiocytic fibroblasticheskoy reaction. At an acute warty endocarditis, or a thrombendocarditis, destruction of an endothelial vystilka with formation of warty trombotichesky imposings on the surface of valves of heart joins it. The fibroplastic (fibrous) endocarditis differs in dominance of a productive component and the strengthened fibrosis of shutters of valves of heart. It can be an outcome of two previous forms. The Vozvratnoborodavchaty endocarditis arises in sclerous the changed and deformed valves at the repeated attacks (a returnable endocarditis) and is shown in the new centers of disorganization, a granulomatosis, damage of an endothelium, trombotichesky imposings and their organization (fig. 4).
Acute warty and vozvratnoborodavchaty endocarditises are found usually at active R., and the volume of trombotichesky imposings corresponds to a degree of activity of process. A diffusion and fibroplastic endocarditis more often happen at low activity of a disease. The acute warty endocarditis can be complicated by a thromboembolism of vessels of a big circle of blood circulation (see. Thromboembolism ).
An outcome of endocarditises is growth of connecting fabric of shutters of valves and fibrous rings of heart, and also the organization of blood clots with the subsequent fibrosis and a hyalinosis of this fabric that conducts to a sclerosis, deformation, an union and low-mobility of shutters of valves of heart. It is morfol. a basis of formation of heart diseases (see. acquired ). The thickening, shortening and an union of tendinous chords which are a consequence of a chordal endocarditis increase deformation and functional inferiority of valves even more. An outcome of a parietal endocarditis (fibroilastichesky, acute warty or returnable and warty) is the focal sclerosis and a hyalinosis of a pristenochny endocardium, is more often than a left ventricle.
Damage of joints at R. at clinically expressed polyarthritis (see) it is morphologically shown in a giperergichesky acute seroznofibrinozny inflammation of a synovial membrane (a synovial membrane of the joint capsule) with an exudate in a joint cavity (see. Synovitis ). The hyperemia, hypostasis, fibrinous treatment and neutrophylic infiltration of a synovial membrane (fig. 5), to a lesser extent the fibrous membrane (fibrous layer), the joint capsule and fabrics surrounding a joint are observed. The inflammation (unlike a pseudorheumatism) does not pass into chronic and does not leave a sclerosis. At absence a wedge, signs of an inflammation of joints in a synovial membrane all phases of disorganization of connecting fabric and other structures, the corresponding activities of rheumatic process, the phenomenon of a vasculitis and capillaritis, nonspecific proliferation of macrophages, fibroblasts, mast cells are observed. Seldom granulemopodobny macrophagic focal proliferata meet. Features of rheumatic damage of joints is smaller, than in heart, depth of disorganization, reversibility of process not only in a phase of mucoid swelling, but also in initial stages of fibrinoid changes, lack of specific granulomas, the progressing sclerosis and hyalinosis.
Defeat of a nervous system. Morfol. changes at R. are observed in all parts of the nervous system. At the heart of changes of c. the N of page lies defeat of vessels of a brain in the form of fibrinoid swelling, is more rare than a necrosis of a wall with a mural thrombosis, proliferation of an endothelium, perivascular limfogistiotsitar-ache infiltrations, sometimes infiltrations of all wall (pan-vasculitis). Small small knots are often formed of accumulation of a microglia with impurity of lymphocytes and plasmocytes. As a result of vascular changes perivascular hypostasis, diapedetic and larger hemorrhages, the small centers of an encephalomalacia develop.
In nervous cells of a brain and nodes of a sympathetic trunk dystrophic and necrobiotic changes, an atrophy are found. Similar changes of cells of a striate body, subthalamic kernels, a cerebral cortex and cerebellum can be the cornerstone of the hysterical chorea meeting more often at children. In a peripheral nervous system the centers of disorganization and focal (diffusion is more rare) cellular infiltration in perineuriums on the course of nervous trunks are found. It leads to eshshevralny and perineural fibrosis.
Defeat of vessels at R. is shown in a combination of all phases of disorganization, a sclerosis and atypical granulomas in an outside cover with cellular infiltration and change of elastic fibers of an average cover. In coronary arteries the centers of a necrosis, a pan-vasculitis and the expressed sclerosis are found; in vessels of kidneys — plasmatic treatment, fibrinoid swelling, proliferation of cells and a sclerosis of an internal cover. Endophlebites are less often observed (see. Phlebitis ) and thrombovasculites (see). Can also arise aortitis (see).
Damage of skin and hypodermic cellulose. In the acute period in a derma sites of disorganization meet, the vasculitis and an endotheliosis, focal inflammatory infiltration are stronger expressed. These changes are noted in externally not changed skin. At a ring-shaped erythema and a purpura in the respective sites the expressed inflammatory infiltration of nipples of a derma and small hemorrhages because of a vasculitis and a capillaritis are observed. The rheumatic small knots which are localized in skin and hypodermic cellulose are characterized by the centers of a fibrinoid necrosis with moderate perifocal makrofagalno - fibroblastichesky reaction, but without the palisadoobrazny arrangement of cells inherent to rhematoid small knots.
Defeat of other bodies and fabrics. In lungs, in addition to a vasculitis, it is noted diffusion or focal, nodular intersticial infiltration of interalveolar partitions mononuclear cells, accumulation in a gleam of alveoluses of eosinophilic proteinaceous membranes (products of exudation) and alveolar phagocytes is more rare. According to N. A. Krayevsky (1941), at considerable expressiveness of these changes there is a peculiar rheumatic pneumonia.
The liver is also involved in rheumatic process. At acute R. signs of a hyperpermeability of capillaries, a vasculitis, fibrinoid changes of vessels and a stroma, polymorphocellular infiltration of a stroma, dystrophy of hepatocytes are noted. In total it can bring to to hepatitis (see) and to sclerous changes in a liver, to-rye amplify at the venous stagnation connected with formation of heart disease.
In kidneys, except the above described changes of vessels of all calibers, can develop acute, and then diffusion glomerulonephritis (see) in connection with defeat of basal membranes of renal balls. In rare instances it leads to development of a secondary contracted kidney.
In closed glands because of disorganization of connecting fabric and a vasculitis interstitial fibrosis with a secondary atrophy of a parenchyma develops. In adrenal glands accumulation of ketosteroids in cortical substance is observed that speaks about its hyperfunction.
In almonds the picture chronic is often noted tonsillitis (see), and capsular almonds the cellular small knots similar to atypical rheumatic granulomas are sometimes formed. In limf, follicles of almonds, limf, nodes and a spleen the expressed plazmokletochny reaction reflecting immune shifts at
R. V skeletal muscles at R.'s aggravation is usually visible the centers of disorganization, a serous and infiltrative and productive interstitial inflammation, and in some cases the granulomas similar (but not identical) with rheumatic granulomas of heart are observed.
Domestic rheumatological school strictly allocates from group of rheumatic diseases true R. (Sokolsky's disease — Buyo), putting in this concept not only acute episodes («acute rheumatic fever»), but also all stages of a course of a disease with her tendency to a recurrence and formation of valve heart diseases. The last option of classification and R.'s nomenclature is accepted on a symposium of All-Union science foundation of rheumatologists in December, 1964. Allocate active and inactive phases of a disease. At the same time the possibility of transition of active rheumatic process to an inactive phase of a disease and division of an active phase on three degrees of activity is acknowledged. As the main component of the concept «activity» first of all inflammatory rheumatic process in bodies and fabrics in its clinical laboratory display is meant. Vkhmeste with that is taken into account that the concept «activity», as well as the concept «disease», is not exhausted only by an inflammation, but by all means includes also elements of damage, dystrophy, multidimensional zashchitnoprisposobitelny, including immune, the answer of an organism. On character of a current allocate acute, subacute, long, continuous re-tsidiviruyushchee and the latent course of rheumatic process. The Klinikoanatomichesky characteristic of damages of heart in an active phase of a disease includes primary rheumatic carditis without defect of the valve, a returnable rheumatic carditis with defect of the valve and without explicit changes from heart, in an inactive phase — a rheumatic myocardiosclerosis and heart disease.
It should be noted that at the modern trend of rheumatism returnable myocarditis sometimes, especially at children's age, can be without heart disease as well as the inactive phase is characterized by lack of changes from heart (a myocardiosclerosis or heart disease). In an active phase of a disease distinguish polyarthritis, serosites (pleurisy, peritonitis, an abdominal syndrome), a chorea, encephalitis, an encephalomeningitis, psychological frustration, nephrite, hepatitis, damage of skin from defeats of other bodies and systems, etc. In an inactive phase the residual phenomena of the extracardiac defeats postponed in an active phase are noted. The short clinical laboratory characteristic of the degrees of activity of rheumatic process allocated in classification is provided in tab. 1.
For the characteristic of features of a current of R. the kliniko-time principle is used, according to the Crimea the active phase of a disease (the rheumatic attack) can have acute, subacute and three options of a chronic current: long, continuous and recurrent and latent (tab. 2).
Thus, working classification allows to estimate R. as pathogenetic a multidimensional disease at the specific patient with differentiation on a degree of activity, character of a current, with the instruction on primary or returnable nature of rheumatic process that considerably helps the doctor with definition of tactics of maintaining the patient and the organization of the individualized dispensary observation.
The MAIN CLINICAL DISPLAYS of RHEUMATISM, ITS CURRENT AND DIAGNOSIS
Despite polymorphism of clinical manifestations of R., in its development are noted the accurate patterns allowing to distinguish a disease as strictly outlined nosological form. In particular, it is necessary to refer R.'s communication with a streptococcal infection to them (existence of the eclipse period between the termination of an infection and initial symptoms of a disease). Are characteristic of R. also a tendency to polisindromno-st of clinical manifestations, existence of standard options of the beginning and the course of a disease, existence in a clinical picture for the first time of the «absolute symptoms of rheumatism» allocated with A. A. Kisel and, at last, R.'s tendency to aggravations and a recurrence. In large part cases classical acute forms P. come to an end with an absolute recovery. At the same time, mainly at a long current and R.'s recurrence, there can be conditions promoting the subsequent transition of a disease to the chronic, torpid, often escalating process.
A. I. Nesterov (1973) allocates three periods in development of rheumatic process. The first (hidden) period proceeds usually 2 — 4 weeks after the termination of a streptococcal infection before initial manifestations of the rheumatic attack. It is characterized by processes of toxi-infectious damage of connective tissue structures and immunological reorganization of an organism in response to streptococcal antigenic influence. Clinically this period proceeds or asymptomatically, or with the manifestations inherent to the period of prolonged reconvalescence. The second period — the period of giperergichesky fabric reactions with clear and characteristic clinical displays of a disease (the rheumatic attack). It is important to note, as against the background of adequate antirheumatic therapy involution of clinical symptoms of the attack does not occur in one step. After disappearance of bright inflammatory displays of a disease its transition to the hidden phase with gradual normalization of immunological and biochemical indicators is natural. The third period is shown by diverse forms of returnable Ruble. It is characterized by a certain tendency to further disturbance and qualitative izkhme-neniye of defense and compensatory reactions with increase of immunological frustration, progressing of dystrophic processes. Clinically much more often the long and continuous and recuring options of disease leading to the progressing circulatory unefficiency and other complications defining a failure
of R. Vydelyayut also primary and returnable Ruble are found. Primary R., as a rule, arises at children's and youthful age, is characterized preferential acute and subacute by options of disease (at 88% of patients, according to 3. P. Annenkova, 1981). Accurate age pattern in primary R.'s development is noted: babies, as a rule, are not ill, two - three-year-old get sick extremely seldom, and in the next years incidence increases, reaching a maximum at children of 7 — 14 years and teenagers. At children's age of usually sexual distinctions in incidence of primary R. it is not observed, among teenagers more often R. get sick female persons.
Fever, the migrating polyarthritis belongs to initial clinical manifestations of acute primary R. (at 50 — 60% of the diseased, on materials of Institute of rheumatology of the USSR Academy of Medical Sciences) or the expressed arthralgias, it is rather rare — a serositis and signs of defeat of other bodies and systems. The interrelation between high fever, polyarthritis and a serositis is noted. The carditis even expressed can proceed only with moderate temperature increase or at a standard temperature of a body. Full and bystry reversibility of polyarthritis and rather favorable course of visceral manifestations of R. under the influence of antiinflammatory therapy is characteristic. High credits of antistreptococcal antibodies and substantial increase of laboratory indicators of activity of an inflammation are defined.
Primary and chronic options of a current of R. are rather rare. There is not specified a question of the specific weight of a primary and latent current in structure of incidence a River.
Generalized exudative inflammatory reactions, however for the last 20 — 25 years are inherent to primary R. (since the end of the 50th years) primary R.'s evolution towards mitigation bright exudative giperergicheskikh forms of a disease is noted. Acute inflammatory defeats at the same time of all covers of heart (pancarditis), primary exudative pleurisy, perikardita, acute meningoentsefalita are rather rare now. Much less often primary rheumatic pneumonia, a ring-shaped erythema, rheumatic small knots come to light, the rheumatic chorea proceeds more softly.
Age features of a current primary R. Tak are noted, at preschool children the long current, and at younger school age — subacute prevails. Constitutes the greatest danger 12 — 14-year age, at Krom more often than in other age groups, also the continuous and recurrent current is found acute and subacute, and sometimes. At the same time in it, as well as at more advanced age (15 — 18 years), the primary and latent current with involvement in process of valves of heart is quite often observed.
Returnable R. as manifestation of the third period of development of a disease is characterized by special tendency to aggravations and a recurrence, the progressing disturbances of a trophicity and regulatory processes with dominance long, nepreryv - but - recurrent options of a current. On materials of institute of rheumatology of the USSR Academy of Medical Sciences (V. A. Nasonova, I. A. Bronzov, 1978), among 200 patients with primary R. acute and subacute disease is noted at 77,5%, continuous and recuring — at 2,5%, and long — at 20%. At the same time among 200 patients with returnable R. these indicators make respectively 28%, 11% and 61%.
It is similar primary, returnable R. can be shown by polyarthritis, involvement in inflammatory process of the serous covers, slight, a nervous system and other bodies. However the most constant is the carditis, the clinical current to-rogo most often defines character of a current of returnable R. in general. At the same time, in process of emergence of new aggravations, along with urezhe-niy frequencies and easing of expressiveness of damage of joints progressive weighting of cardiac pathology due to increase of defeat of valves and a myocardium of heart is noted. Considerably the continuous and recurrent current meets more often.
A rheumatic carditis (rheumatic carditis)
the Rheumatic carditis — the leading manifestation of active R. defining weight of a current and the forecast of a disease. The tendency to consecutive or simultaneous inflammatory damage of a myocardium, an endocardium and a pericardium belongs to features of a rheumatic carditis (see the Pancarditis). Due to the difficulties of differentiation of defeat of separate covers of heart in clinical practice the generalized term «rheumatic carditis» was widely adopted.
Primary rheumatic carditis, according to different researchers, is distinguished at 80 — 90% of children and at 95 — 100% of adult, sick Rubles. Despite existence of nek-ry age features, basic distinctions in clinical manifestations of primary rheumatic carditis at children, teenagers and adults are not marked out. The symptomatology of a rheumatic carditis in many respects is defined by preferential defeat of this or that cover of heart — a myocardium, an endocardium and a pericardium. Significant effect on the characteristic of a rheumatic carditis is had expressiveness of changes from heart, degree of the general activity of rheumatic process and character of a course of a disease.
Rheumatic myocarditis is the basic, and is frequent also the only symptom of a disease. So, at 40 — 60% of sick R. at the beginning of a disease (especially at an acute current) changes from heart keep within a picture of the isolated myocarditis (see) which is morphologically varying from diffusion to focal. Diffusion myocarditis meets pronounced clinical manifestations in 70 — the 80th years less than 20 — 30 years ago, and it is observed preferential at children of 12 — 14 years and teenagers. Also poorly expressed myocarditis is prevailing moderately. At patients with rheumatic myocarditis disturbance of the general state, weakness, an asthma, cardialgias, heartbeat are quite often noted. Expressiveness of subjective unpleasant feelings usually small therefore they come to light at careful purposeful poll of patients more often. Change of heart rate in rest belongs to early objective symptoms — tachycardia (see), bradycardia (see) and respiratory arrhythmia of heart (see); increase in the sizes of heart (from insignificant to expressed), easing or dullness of cardiac sounds (see), emergence of a three-membered cordial rhythm. On the phonocardiogram usually find decrease in amplitude of the I tone, its deformation, splitting and broadening, so-called pathological III also are more rare the IV tones (high-amplitude, written down in an average and high-frequency range, with the wide check-in area), merge to-rykh creates a clinical picture of a summatsionny cantering rhythm (see. Gallop rhythm ).
Practically all patients with rheumatic myocarditis have different degree of manifestation a systolic noise with epicenter in the V point (the IV mezhreberye to the left of a breast) and in the field of a pulmonary trunk. This noise registers in FKG in the form of the mid-frequency and sredneamplitudny mesosystolic noise of the ovate-abating form borrowing 1/2 — 1/3 systoles. At a part of patients in a zone of a projection of the mitral valve mesodiastolic noise can be registered. Dynamism and variability of the specified symptoms, especially under the influence of antiinflammatory treatment is characteristic of rheumatic myocarditis.
On an ECG at rheumatic myocarditis dysfunction of a sinus and atrial node (tachycardia, bradycardia, a respiratory arrhythmia) quite often is registered, disturbances of a rhythm come to light less often: atrioventricular dissociation with an interference (fig. 6), migration of a pacemaker in auricles and an atrioventricular node (fig. 7), is possible premature ventricular contraction. Along with it on an ECG disturbances of conductivity in the form of an atrioventricular block of I can be noted and the II degrees (fig. 8) are much more rare. Along with absolute specific elongation of an interval of PQ quite often is found. In similar observations the size of an interval of PQ corresponding at the first inspection to normal values (0,20 sec.) in the course of treatment decreases by 0,05 — 0,06 sec. without essential changes of heart rate. Are most frequent at rheumatic myocarditis of disturbance of bioelectric processes in a myocardium of ventricles. It finds reflection in change of a tooth of T, pathological shift (is more often down) a segment of ST and lengthening of an electrical systole. Changes of a tooth of T are expressed in moderate decrease in its amplitude and broadening, is more rare in flattening to the isoelectric line, certain patients have negative teeth of T. U of the same patient various electrocardiographic changes which are characterized by dynamism and reversibility can be observed.
At radiological inspection using rentgenokimografiya (see) and elektrokimografiya (see), according to V. A. Shanina (1968) and E. S. Lepskoy (1980), at the vast majority of patients with primary rheumatic carditis signs of decrease in sokratitelny and tonic functions of a myocardium (disturbance of character of a pulsation, change of amplitude and a form of teeth of rentgenokimografichesky and elektrokimografichesky curves), the inadequate answer — increase of cordial reductions at decrease in depth of a pulsation on an exercise stress come to light. Less constantly (only at the expressed myocarditis) the sizes, a configuration and position of heart change.
For assessment of a condition of sokratitelny function of a myocardium the echocardiography has additional value. At most of patients during the acute period of a disease signs of a giperkinrtichesky condition of blood circulation (increase in shock and minute volumes) and slightly raised or normal indicators of sokratitelny function of a myocardium (fraction of exile, degree and speed of shortening of diameter of a left ventricle) are noted. Reliable decrease in these indicators is observed only at the expressed myocarditis.
In the conditions of active treatment of rheumatic myocarditis the prevailing most of patients (59%) practically recovers (there are no changes from heart). At a part of patients signs of miokardichesky come to light cardiosclerosis (see), the frequency of development to-rogo with age patients increases.
A rheumatic endocarditis
B 70 — the 80th years clinical signs of involvement in process of valves of heart at the first attack of R. at children come to light at 50 — 55% of patients. The tendency to development of a valvulitis — diffusion defeat of deep layers of valves is characteristic of children's age. At the prevailing most of patients the valvulitis is combined with damage of a myocardium (endomyocarditis). Both at children's age, and at adults signs of defeat of valves of heart can appear from the first days of the rheumatic attack.
According to observations of pediatricians-rheumatologists of D. D. Lebedev (1951), 3. I. Edelman (1962), A. B. Volovika (1965), H. M. Kurenskoy (1975), A. V. Dolgopolova in recognition of defeat of valves of heart clinical signs have (1977) conducting value. Emergence of systolic noise of various intensity with the characteristic «blowing» shade is a constant sign of defeat of the mitral valve, to-ry it is best of all listened in a zone of a projection of the mitral valve (a top of heart, the V point) in a prone position and on the left side, quite often carried out to the left, to the axillary area, and amplifies after an exercise stress. On the phonocardiogram the specified noise registers in a look highly - and mid-frequency, pansystolic or protosystolic noise, the small or average amplitude abating or a tape-like form with epicenter of registration at a top of heart (fig. 9). Such qualitative characteristic allows to connect it with defeat of mitral valve (see. acquired ).
The specified systolic noise against the background of subsiding of process is characterized by dynamism, gradually changing on amplitude, a form and duration. At most of patients systolic noise gradually decreases on amplitude and duration. At nek-ry patients it at first amplifies, becomes more high-frequency, then slowly weakens. At a part of patients systolic noise remains long, high-frequency. Along with systolic noise in the field of a top of heart at nek-ry patients with a valvulitis of the mitral valve it is listened mesodiastolic low - and the mid-frequency noise of small amplitude which is following immediately the III tone, quickly fading. Mesodiastolic noise is registered on the phonocardiogram more often, than is defined auskultativno.
Dynamic changes of the sizes of heart reckon with expansion of its borders preferential to the left as a characteristic sign of a valvulitis of the mitral valve. At X-ray inspection note emergence of a so-called mitral configuration of heart due to performance of a waist of heart by an ear of the increased left auricle, expansion of the sizes left an auricle and a ventricle (fig. 10). According to E. Lensk (1980), more than at 1/3 patients disturbances of a hemodynamics in a venous part of a vascular bed of lungs are noted that radiological is characterized by relative expansion of pulmonary veins. At an electrocardiographic research at 2/3 patients preferential with a pronounced carditis emergence of signs of an acute overload of the left auricle with a mitralization of teeth P is noted (broadening of a tooth P electrocardiograms> 0,10 sec.; its two-staging in the right chest assignments with dominance of a negative phase). According to N. Artamonova (1977), at 1/3 patients these changes are combined with initial symptoms of increase in a left ventricle.
Defeat of valves of an aorta is characterized by emergence of the «flowing» diastolic noise defined along the left edge of a breast and registered on the phonocardiogram right after the II tone in the form of a protodiastolic high-frequency noise. The specified noise is listened better, than registers in FKG. On an ECG at a valvulitis of valves of an aorta signs of a diastolic overload of a left ventricle quite often are registered. Radiological the tendency to horizontal position and a passing aortal configuration of heart, preferential increase in the sizes of a left ventricle, relative strengthening of a pulsation of a left ventricle and an aorta is characteristic. At primary rheumatic carditis perhaps simultaneous defeat of the mitral valve and valves of an aorta.
At recognition of a rheumatic valvulitis the method is of a certain diagnostic value echocardiography (see).
At most of patients with defeat of the mitral valve on an ekhokardiogramma the thickening, «lokhmatost» of an echo signal from shutters of the valve (or tendinous chords), increase in amplitude of opening of its front shutter comes to light; deformation of a systolic fragment of the mitral valve in the form of its deflection and more horizontal direction, than normal is less often observed; sometimes immobilization of a back shutter of the valve (fig. 11) is defined. Quite often these changes are combined with clear dilatation of the left auricle and a left ventricle, sometimes with a hyperkinesia of its walls. At defeat of valves of an aorta more than melkoamplitudny trembling of a lobby (perhaps, and back) shutters of the mitral valve is found in a half of patients that in total with dilatation of a left ventricle, a hyperkinesia of its walls testifies to aortal regurgitation. A mitral and aortal valvulitis meet at the acute and long course of rheumatism more often.
As show long observations, an outcome of primary rheumatic valvulitis in the conditions of its modern trend and active pathogenetic treatment approximately at a half of patients is practical recovery, at other half of patients formation of valve heart disease is noted. The percent of an outcome of primary rheumatic carditis in heart disease at teenagers is much higher, than at children and adults. The most frequent heart disease after the postponed valvulitis at children is insufficiency of the mitral valve, insufficiency of the aortal valve and as an exception — the isolated mitral stenosis or the combined mitral defect is more rare. In modern conditions the heart diseases created after primary rheumatic carditis are characterized by a number of features. Unlike last years in 70 — the 80th rate of formation of heart disease changed. Signs of the last at most of patients are shown in the acute period of a disease, remain in poslepristupny, however are made out in a clear picture of heart disease only within the next 2 — 3 years. The heart diseases created after primary rheumatic carditis are characterized by unsharp expressiveness and permanent compensation for a row of years. According to N. I. Alexandrova (1979), at persons at the age of 16 — 18 years unlike children already in the result of the attack the combined mitral defect quite often forms.
The rheumatic pericardis
the Rheumatic pericardis develops much more often than it is diagnosed. So, clinically the pericardis in modern conditions at the first attack of a disease is distinguished only at 1,0 — 1,5% of children, is slightly more often at youthful age and at adults while, according to V. A. Shanina (1962) and E. S. Lepskoy (1980), careful X-ray inspection at primary rheumatic carditis allows to reveal defeat of a pericardium at 40% of children and at 51,1% of adult patients, is preferential in the form of the plevroperikardialny changes arising on process of a disease. From the kliniko-anatomic point of view distinguish fibrinous and exudative (serofibrinous) pericardis (see). Unlike last years in 70 — the 80th the rheumatic pericardis at children, teenagers and adults is more often shown in the form of limited and unstable adhesive process.
The shaggy pericardium at most of patients proceeds with unsharply expressed clinical symptomatology. Emergence of a pericardial rub is the most reliable sign, to-ry it is characterized by various expressiveness, localization and duration of listening. Radiological the adhesive pericarditis in early stages is expressed by loss of clearness, unsharpness of outlines of contours of a cordial shadow on the limited site, later — emergence of roughness, an izlomannost, ugloobrazny pla of parusoobrazny deformation of contours of heart due to formation of plevroperikardialny commissures. On a rentgenokimogramma according to area of defeat the multicircuit curve with unsharply outlined deformed teeth is registered. The most constant is localization of adhesive process in a pericardium of area of a top of heart and in the area adjoining the left part of a dome of a diaphragm.
The exudative pericardis is quite often combined with damage of a myocardium and the valve device of heart (pancarditis) and shown by deterioration in the general state, poyavleni-niy an asthma and retrosternal pains, tachycardia and other symptoms of a circulatory unefficiency considerably accruing in horizontal position of the patient. Per-kutorno also radiological is marked out expansion of the sizes of heart, the zone of absolute dullness of heart increases, the shadow of heart gains triangular shape. On a rentgenokimogramma accurate decrease in amplitude of cordial reductions comes to light. Cardiac sounds and noise weaken, pulse becomes frequent, small, arterial pressure decreases, venous — swelling of cervical veins raises, found.
On an ECG characteristic dynamics of changes is noted. In an initial stage the shift of a segment S T up from the isoline and increase in teeth of T is defined. Further there is a gradual return of a segment of ST to the isoline, decrease in a tooth of T and its transition to negative. In a phase of recovery the tooth of T is returned to a normal amount and a form. In identification of a pericardiac exudate the echocardiography has great diagnostic value. Emergence so-called an echo free space around heart is characteristic («stratification» of an epicardium and pericardium in the field of a back wall of a left ventricle; «peeling» of a front wall of a right ventricle from a chest wall), decrease in amplitude of the movement of a pericardium.
The conventional differential and diagnostic difference of a rheumatic pericardis is bystry disappearance of its main symptoms under the influence of active antirheumatic treatment, a cut allows to prevent at the prevailing most of patients formation of commissures and an obliteration of a pericardiac cavity.
The diagnosis of primary rheumatic carditis, criteria for evaluation of degree of its expressiveness, the differential diagnosis
the Diagnosis of primary rheumatic carditis is based, on the one hand, on reliable active R.'s establishment, with another — on identification of a characteristic cardiovascular syndrome. The major diagnostic importance has a combination of the following signs: preferential developing of a disease at children's and teenage age; close connection of a disease with the previous nasopharyngeal infection; existence of an interval (2 — 4 weeks) between the termination of a nasopharyngeal streptococcal infection and the beginning of a disease, is more rare — prolonged recovery after a nasopharyngeal infection; frequent fervescence at the beginning of a disease, joint pains and development of polyarthritis; emergence of cardial complaints, fatigues, weaknesses, auskultativny and phonocardiographic signs of a valvulitis of the mitral valve and (or) valves of an aorta; electrocardiographic data (emergence sinus takhi-or bradyarrhythmias, disturbances of a rhythm — atrioventricular dissociation with an interference, migration of a pacemaker, delay of atrioventricular conductivity, change of a tooth P, changes of a tooth of, the shift of a segment of ST, lengthening of an electrical systole of QT); radiological changes (emergence of signs of disturbance of sokratitelny and tonic function of a myocardium, expansion of borders of a cordial shadow at the expense of a left ventricle or left an auricle and a ventricle of heart, signs of involvement in process of a pericardium); emergence of symptoms of a circulatory unefficiency; dynamism and variability of clinical and functional indicators under the influence of active antirheumatic therapy.
Establishment of degree of manifestation of cordial changes at primary rheumatic carditis since it allows to estimate weight of damage of heart is extremely important for clinical rheumatology, to appoint adequate pathogenetic treatment and to hold in dalneyshekhm all necessary therapeutic and preventive actions. A. I. Nesterov (1969, 1973) suggested to be guided by three most important gradation of a rheumatic carditis in the practical relation (bright, moderately and poorly expressed). For the differentiated assessment of expressiveness of a rheumatic carditis the wide complex modern clinical, tool graphical methods of a research, and also mathematical and cybernetic receptions is used. In 70 — the 80th at the prevailing most of patients (75 — 80%) also poorly expressed rheumatic carditis comes to light moderately. The pronounced rheumatic carditis meets at children's age more often.
The group with a pronounced rheumatic carditis is made by patients, at to-rykh the tendency to simultaneous defeat of several covers of heart is noted. Characteristic feature of this option of a rheumatic carditis is disturbance of the general condition of patients. Objective signs of damage of heart are shown clearly that finds reflection in demonstrative clinical, tool and graphic and laboratory symptomatology. The most informative symptom complex of a pronounced rheumatic carditis developed by means of mathematical receptions includes: the considerable expansion of percussion borders of heart (in both parties) confirmed radiological by increase in several cameras of heart (a left ventricle, the left auricle and a right ventricle) or expansion of percussion borders of heart to the left (on 2 cm and more) that radiological corresponds to increase in a left ventricle (the II—III degree on classification And. X. Rabkin and G. M. Savelyeva); the considerable muting of cardiac sounds registered on the phonocardiogram in the form of the expressed easing (decrease in amplitude) and deformation of the I tone (at normal atrioventricular conductivity); pericardial rub; listened at the same time or separately the blowing systolic noise on a top (high-frequency, pansystolic on the phonocardiogram) and the «flowing» diastolic noise in the V point (high-frequency, protodiastolic on FKG); mesodiastolic noise; considerable changes of a tooth of T (decrease, broadening, deformation) on an ECG; tachycardia; pathological III and IV tones; delay of atrioventricular conductivity, circulatory unefficiency of the II stage. Existence of the specified complex of signs or several of them in any combination to other symptoms of damage of heart allows to regard primary rheumatic carditis as pronounced on kliniko-tool manifestations. Echocardiographic this option of a rheumatic carditis is characterized by clear dilatation of a left ventricle, the left auricle, is more rare increase in the sizes of a right ventricle. Existence of a valvulitis of the mitral valve or valves of an aorta causes the corresponding qualitative and quantitative changes of an echogram from the mitral valve or valves of an aorta (see above the Rheumatic endocarditis). At the same time disturbances of sokratitelny function of a myocardium of a left ventricle come to light. The pronounced rheumatic carditis is quite often combined with noncardiac manifestations of R. (preferential polyarthritis) and considerable changes of laboratory indicators of activity of inflammatory process. This option of a rheumatic carditis develops preferential at patients with an acute current and is characterized by accurate dynamism of all manifestations.
Moderately expressed rheumatic carditis is noted at defeat preferential by one, is more rare than two covers of heart (myocarditis or endomyocarditis without clear signs of defeat of valves). Objective signs of a rheumatic carditis in this case are characterized by smaller demonstrativeness.
The most informative indicators of moderately expressed rheumatic carditis are: the expansion of percussion borders of heart to the left (on 1 — 1,5 cm) confirmed radiological by increase in a left ventricle (the II degree); clear systolic noise on a top and in the V point, written down on the phonocardiogram in the form of the mid-frequency, sredneamplitudny, ovate-abating noise, is possible the systolic noise of the blowing character on a top registered on the phonocardiogram in the form of low-amplitude, high-frequency, about-tosistolichesky or pansystolic noise; the moderate muting of cardiac sounds registered on FKG in the form of unsharp decrease in amplitude of the I tone; pathological III tone; the signs of an adhesive pleuropericarditis revealed radiological; disturbances of a rhythm, disturbance of atrioventricular conductivity (less often and less expressed, than at a pronounced rheumatic carditis) and lengthening of an electrical systole, defined on an ECG. Echocardiographic at this option of a rheumatic carditis moderate dilatation of the left cameras of heart (a left ventricle, the left auricle) comes to light. At a number of patients nek-ry qualitative and quantitative changes of an echogram from the mitral valve can be defined, at others change of sokratitelny function of a myocardium of a left ventricle is noted. During the subsiding of rheumatic process quite bystry positive dynamics of echocardiographic changes, as a rule, comes to light. Moderately expressed rheumatic carditis often is combined with noncardiac manifestations of R., observed at maximum and moderate, is more rare than the minimum degree of activity, is preferential at a subacute current
of R. Simptomatik of poorly expressed rheumatic carditis more «modest». Treat the most informative signs of this option: disturbance of heart rate (tachycardia and bradycardia); insignificant expansion of borders of heart to the left (0,5 — 1,0 cm), not always confirmable radiological; the insignificant muting of cardiac sounds registered on the phonocardiogram in the form of unsharp easing of the I tone (decrease in its amplitude); weak also is more rare the clear systolic noise registered on the phonocardiogram is preferential in the form of mid-frequency, mesosystolic noise of average amplitude and duration; the disturbance of atrioventricular conductivity and lengthening of an electrical systole defined on an ECG; the combination of a rheumatic carditis to polyarthritis or a chorea is frequent. On an ekhokardiogramma at this option of a rheumatic carditis of change, as a rule, do not come to light, however at a part of patients increase in shock and minute volumes of blood circulation is defined a nek-swarm. Poorly expressed rheumatic carditis can be observed at all options of a current of R. and various degree of its activity.
The differential diagnosis at primary rheumatic carditis is carried most often out with not rheumatic myocardites and so-called functional cardiomyopathies (see). Unlike primary rheumatic carditis not rheumatic myocardites (see) have the clinical symptomatology and are characterized by a number of features: existence of the previous allergic diseases, quite frequent and close connection with the postponed, preferential viral nasopharyngeal infection, with the subsequent short «light» interval, abundance and persistence of cardial complaints, considerable changes on an ECG, the minimum shifts of laboratory indicators of activity of an inflammation, bystry development of an astenpzation, slow positive dynamics of clinical and electrocardiographic changes under the influence of therapy.
Functional cardiopathies are characterized by the following manifestations: existence of the chronic centers of an infection (is more often at children), the previous vegetative and endocrine dysfunction (is more often at adults), frequent communication of a disease with various stressorny influences, existence of asthenoneurotic emotionally charged «cardial complaints», lack of objective symptoms of cardial pathology, existence of periodically arising vegetovascular crises (is more often at teenagers and adults), lack of laboratory signs of inflammatory activity, clear effect of sedative drugs.
N. N. Kuzmina and T. G. Glazkova (1975) developed by means of the COMPUTER the so-called decisive rule for differential diagnosis of primary rheumatic carditis and similar diseases (not rheumatic myocardites, functional a cardiomyopathy) at children's age. The rule has an appearance of the table consisting of the list of signs, against each of to-rykh costs information coefficient. On the basis of results of comprehensive examination of the patient make algebraic addition of coefficients of the signs which are available for this patient for its practical application and compare the received sum to the threshold value presented at the end of the table. If the received sum is more than threshold value, then primary rheumatic carditis is diagnosed for the child. Reliability of diagnosis of primary rheumatic carditis by means of this table makes 94,3%.
The returnable rheumatic carditis is most often observed at adults and teenagers, however meets also at children's age. This option of a rheumatic carditis usually develops against the background of a miokarditichesky cardiosclerosis, and is frequent also the created heart disease. The clinical picture of a returnable rheumatic carditis is characterized by a symptom complex of recurrent myocarditis and signs of recurrent valve defeat. As a recurrence of a rheumatic carditis arises against the background of already broken trophicity and decrease in functional reserves of a myocardium, in their symptomatology quite often into the forefront signs act circulatory unefficiencies (see), to-rye especially are expressed and inclined to increase, than more was a recurrence of a rheumatic carditis in the past and than heart disease is heavier, and also than the patient was less persistently treated. At a recurrence of a rheumatic carditis at teenagers and young men defeat of two valves quite often comes to light (mitral and aortas), at patients 30 years are more senior more often defeat of one valve is observed.
Emergence of new noise or increase of their intensity, change of sonority of cardiac sounds is characteristic. At the subsequent recurrence of a rheumatic carditis deformation of valves progresses and the clinical picture of heart disease becomes more expressed, quite often there is its complication (transformation of insufficiency of the mitral valve in the combined mitral defect or emergence of new heart diseases). The accruing circulatory unefficiency becomes one of the leading manifestations of recurrent rheumatic process in heart. Characteristic manifestations of the returnable rheumatic carditis proceeding against the background of a miokarditichesky cardiosclerosis (see) and myocardial dystrophies (see), disturbances of a cordial rhythm in the form of premature ventricular contraction are (see), Bouveret's disease (see) or ciliary arrhythmia (see). The returnable rheumatic carditis is characterized by a tendency to a chronic current, poorly expressed exudative component of an inflammation, soft system displays of rheumatism. At a number of patients widespread giperergichesky reactions take place, involvement in process of many bodies and systems is noted, the most adverse forms, the continuous and recurrent course of a disease inherent in preferential returnable R. The returnable rheumatic carditis at an acute, subacute and continuous and recurrent current of R. keeps lines inherent to these options, however is characterized by more considerable weight of damage of heart in connection with existence of a miokarditichesky cardiosclerosis or already created defect. The returnable rheumatic carditis at long option of a current of R. is followed by far come defeat of the valve device of heart and myocardium more often, is characterized by the gradual beginning of a recurrence, a weak and variable effect of antirheumatic therapy, tendency to a steady decompensation of cordial activity. In case of latent option of a current of R. it is characterized by progressing of cordial changes in the absence of clinical symptoms of active rheumatism.
The most frequent source of diagnostic mistakes at recognition of a returnable rheumatic carditis are bacterial endocarditis , (see), heavy options of a course of myocarditis like Abramov — Fidlera (see. Myocarditis ), damage of heart at diffusion diseases of connecting fabric (see. Collagenic diseases , Lupus erythematosus ), nek-ry options of inborn pathology of heart.
One of the main manifestations of R. and one of the main diagnostic criteria of this disease is rheumatic polyarthritis. In a basis polyarthritis (see) at R. lies acute or subacute synovitis (see), to-ry it is characterized by multiple damage of large and average joints (coxofemoral, knee, talocrural, elbow) and is much more rare — small joints, brushes and feet. Main symptoms of polyarthritis: sharp pains, swelling and deformation, restriction of mobility of joints because of pains, temperature increase of skin over joints, and at a number of patients — erubescence over the affected joints. Instability of inflammatory changes, their bystry transition from one joint to another («flying rheumatism»), symmetry of defeat, bystry complete elimination of all painful phenomena within several days are characteristic. At a part of patients can it is long to remain small arthralgias (see), amplifying at a weather changing, cooling, under the influence of acute respiratory diseases. Rheumatic polyarthritis is followed by considerable changes of laboratory indicators of activity of an inflammation and corresponds to usually maximum degree of activity of R. Imeyutsya of the instruction on possibility of permanent deformations of preferential small joints of brushes — the so-called chronic post-rheumatic arthritis for the first time described to S. Jac-coud in 1869. Refer ulnarny deviation of brushes in a combination to flexion contractures of metacarpophalangeal joints to its features, weak expressiveness or total absence of a pain syndrome, lack of erosive changes in bones and rhematoid factor (see) in blood serum.
Differential diagnosis of rheumatic polyarthritis is carried out with infectious arthritises (see Arthritises, Infectious allergic polyarthritis), pseudorheumatism (see), a system lupus erythematosus (see), scleroderma (see), serum disease (see), a hemorrhagic vasculitis (see. Shenleyna — Genokh a disease ) also it is, as a rule, simple owing to simultaneous existence at the listed diseases of other characteristic clinical and laboratory manifestations, tendency to heavier and long current of a joint syndrome. Great difficulties arise during the carrying out the differential diagnosis of rheumatic polyarthritis with postinfectious reactive arthritises, in particular yersinia arthritis (see. Iyersinioz ), with palindromny rheumatism (see), Lefgren's syndrome (see. Lefgrena syndrome ), with polyarthritis at a leukosis (see. Leukoses ).
Defeat of a nervous system
according to V. V. Mikheyev (1960), defeat of a nervous system at R. takes on frequency the third place after damage of heart and joints. Founder of the doctrine about a nervous form of rheumatism Hervé de Chegoin (N. - J.Hervez deChegoin) in 1845 described several cases of rheumatic damage of a brain with mental disorders, having offered the term «brain rheumatism». In the researches conducted by hl. obr. the Soviet scientists V. K. Beletsky and A. P. Avtsyny (1939), V. V. Mikheyev (1949, 1971), N. B. Mankov-sky (1959), M. B. Tsuker (1978), etc., versatily studied clinic and a patomorfologiya of defeats of the central and peripheral nervous system at rheumatism. In 60 — the 70th the term «neurorhematism» offered by V. V. Mikheyev (1960) began to be applied to designation of nervous forms P. In development of a neurorhematism inflammatory changes of vessels of a nervous system have major importance (vasculites). Also an opportunity direct toksiko-aller-gicheskogo and immunopathological impact on nervous structures is not excluded.
The standard classification of a neurorhematism it is not developed. In N. B. Mankovsky's classification (1959) four main forms of a neurorhematism are allocated: hysterical chorea, acute encephalomeningitis, chronic encephalomeningitis, rheumatic encephalopathy. From the middle of the 70th the clear tendency to reduction as frequencies of defeat of a nervous system is observed at rheumatism, and expressiveness of clinical manifestations of a neurorhematism.
Hysterical chorea. In 1686 Sydenham offered the term «hysterical chorea». In 1891. A. A. Kisel entered a hysterical chorea into the absolute symptoms of rheumatism formulated by it. V. V. Mikheyev (1960), M. B. Tsuker (1963) carry a hysterical chorea to rheumatic encephalitis with preferential defeat of striopallidal system. The hysterical chorea develops at 11 — 13% of the children who got sick with R. at the age of 6 — 15 years is more often (in 60 — 70% of cases) during the first attack of a disease. Girls are ill more often than boys. With approach of puberty the hysterical chorea arises less often.
Clinical manifestations choreas (see) develop gradually. At first there are signs of astenisation, unstable mood, irritability, absent-mindedness, tearfulness, a bad dream, progress of the child worsens. In 8 — 10 days the main symptoms of a hysterical chorea, first of all hyperkinesias (the involuntary, disproportionate, in-coordinate gusty movements) in a combination to decrease in a muscle tone join. Hyperkinesias are noted in face muscles, necks, extremities, a trunk. Grimacing is observed, hands, legs, muscles of a trunk randomly «dance». It is difficult for child to bring a spoon to a mouth, it is difficult to eat and drink independently. His speech becomes not clear, is followed by «proglatyvaniye» of separate syllables and the whole words. Hyperkinesias amplify at nervousness, disappear during sleep. More often they are bilateral, but are sometimes observed only in one half of a body (hemochorea). In especially hard cases «the motive storm» is observed, at the same time the sharp violent movements do not allow the child to fall asleep, he quickly grows thin. In 70 — the 80th the heavy current of a hysterical chorea is observed seldom, the atypical, erased forms are more often noted, to-rye not always in time are diagnosed. The erased forms of a hysterical chorea are observed preferential at preschool and younger school age and quite often accept a long, wavy current.
Often symptoms of vegeto-vascular dystonia — vasculomotor frustration, a resistant red dermographism (see), the increased perspiration, tachycardia, lability of pulse, sometimes subfebrile temperature, and also disturbances accompany a small chorea from the mental sphere — depression of mood, tearfulness, sensitivity, an indiscipline, scattered attention, fears; more rare, on the contrary, hypererethism, euphoria, hallucinations (see), is more often than frightening contents. In general the combination of the following symptoms (a so-called pentade of a hysterical chorea) is peculiar to a small chorea: a choreic hyperkinesia in more or less sharp form; muscular dystonia with dominance of hypotonia, edge in nek-ry cases is expressed very sharply (during the weakening of a hyperkinesia), up to flabbiness of muscles that gives the grounds to speak about «a soft chorea» (chorea mollis), sometimes even imitating paralyzes («a paralytic chorea»); the statokoordinatsionny disturbances which are especially noted at purposive movements and during the walking («looseness» of gait); disturbances from the autonomic nervous system (the phenomenon of vascular dystonia); psychopathological phenomena.
For specification of the diagnosis, especially at the atypical, erased chorea, resort to a nek-eye, to clinical tests, simple on the performance; from them the most reliable is the following: test Kherson — the child, having narrowed eyes, cannot hold long put out tongue (norm 15 sec.); Filatov's test — at handshake of the patient the doctor feels irregularity of movements; test Tsuker — the patient sits or costs opposite to the doctor, closely touching it with knees, the doctor holds hands of the patient in the hand and talks to him, catching even insignificant hyperkinesias; Iogikhes's test — the patient tries to copy the movements of a hand of the doctor an outstretched arm, at the same time in it the violent movements come to light. For a hysterical chorea change of knee jerks or in the form of hardening of a leg in a pose of extension — a Gordon's sign-2 is pathognomonic (see. Gordon reflexes ), or in the form of pendulum movements of a shin.
As a rule, the hysterical chorea is not followed by the expressed changes of laboratory indicators of activity of an inflammation. Only at very heavy options of a current or accession of a rheumatic carditis (the expressed rheumatic carditis is observed seldom) there are their deviations from normal amounts. On the electroencephalogram (see. Elektroentsefalografiya ) inconstancy of an alpha rhythm, existence of slow waves of various duration and bystry fluctuations like beta waves is noted. Degree of manifestation of these changes correlates with sharpness, weight and duration of a disease, however they are not specific to a chorea.
Average term of a current of a hysterical chorea 2 — 3 months. The long and erased forms, to-rye come to light considerably now more often than acute, proceed sometimes up to one year and are inclined to a recurrence. But even after one attack neurologic disturbances at nek-ry children can come to light within 2 — 3 years in the form of an asthenic syndrome with a bystry exhaustion of cortical processes, change of behavior of the child with tendency to the hysterical reactions easy for disturbances of a craniocereberal innervation, asymmetry of tendon jerks. Single patients after a hysterical chorea have permanent organic lesions of a brain.
Chronic encephalomeningitis. Hypothalamic disturbances most often occur among displays of a chronic rheumatic encephalomeningitis. The clinical picture of hypothalamic disturbances develops usually gradually against the background of the long, recurrent course of rheumatic process. Signs of functional frustration of c can serve as their initial manifestations. N of page (vegeto-vascular dystonia, neurotic, isteroidny reactions, headaches, etc.). Only afterwards into the forefront signs of defeat of hypothalamic area act (see. Hypothalamus ). Most often at the same time paroxysmal vegetovascular frustration of the sympathoadrenal, vagoinsulyar-ny or mixed type are observed. The clinic of hypothalamic disturbances at R., in addition to the specified paroxysms, is characterized by dysfunction of hemadens (Itsenko's syndrome — Cushing, an adiposagenital syndrome, disturbances of a menstrual cycle, not diabetes mellitus, etc.), more rare neurotrophical disturbances, and also disturbances of thermal control, a dream, wakefulness, etc. The course of hypothalamic frustration at R. differs in persistence and duration, correlation of aggravations with aggravations of rheumatic process. At the persons suffering from R. more often than in population, epileptiform attacks meet. They are observed usually against the background of an inactive phase of rheumatism, antirheumatic therapy has no significant effect on the frequency of their emergence.
Symptoms of rheumatic encephalopathy are observed, according to V. A. Nasonova and I. A. Bronzov (1978), more than at a half of patients with long and continuous re-tsidiviruyushchim R. Harakterna's current the various, unsharply expressed central frustration of a somatic and vegetative innervation. Clinically it is shown by headaches, decrease in memory, attention, symptoms of vegetative, emotional lability with disturbance of thermal control, serdtsebiyeniye, vasculomotor frustration, passing disturbances from craniocereberal (cranial, T.) nerves. Symptoms of encephalopathy tend to increase in the period of R.'s aggravation and considerably are exposed to involution during the subsiding of activity of a disease.
Other defeats of a nervous system. The described earlier rheumatic meningitis, arachnoidites, steam-kinsonopodobnye of state, rheumatic damages of a spinal cord now (70 — the 80th) are extremely rare. Considerably also the frequency of detection of the neuritis, polyradiculoneurites and other defeats of a peripheral nervous system which are authentically connected with rheumatism decreased.
Seldom also the hemorrhagic and ischemic strokes described earlier at patients with acute R. caused by a vasculitis of large vessels of a brain meet. At the same time in connection with lengthening of life expectancy of patients with rheumatic heart diseases thromboembolisms of brain vessels began to be noted slightly more often (see. Thrombosis , Thromboembolism ), major importance in development to-rykh plays a circulatory unefficiency.
the Mental disturbances caused by R. meet now are extremely seldom and usually limited to reversible neurosis-like disturbances. The leader at the same time is the asthenic syndrome (see) with the phenomena of irritable weakness, an exhaustion. Patients note increased fatigue, decrease in working capacity, intolerance of bright light, noise, emotional lability, instability of mood are inherent to them.
During the weighting of a state the sleep is interrupted, persistent headaches are possible. The asthenic syndrome can include phobias (see. Persuasive states ), to-rykh the vital fear in connection with change of corporal feelings is the cornerstone. The maintenance of phobias is limited preferential to fears for life, for heart, they can be followed by tachycardia, difficulty of breath, perspiration, a shiver in a body, feeling of a prelum in a breast. The adynamy at R. in some cases is followed by also hypochiondrial frustration (see. Hypochiondrial syndrome ). However the hypochiondrial symptomatology is not so characteristic of sick R. U of patients with the expressed heart diseases against the background of a serious somatic condition astheno-depressive episodes with vegetative lability, and also short-term psychoses with disturbance of consciousness and gallyutsinatornobredovy frustration can develop. In their origin, however, disturbances of blood circulation, a hypoxia, metabolic disturbances, but not actually rheumatic process have major importance. The depression amplifies by the evening. The described earlier so-called acute rheumatic psychoses with manifestations in the form of a delirium, delirious and amental, delirious oneyroidiogo, hallucinatory paranoid syndromes with episodes of not clear consciousness, a stupor, apathetic, hallucinatory and amental states, to-rye can replace each other, now practically do not meet.
Damages of lungs
Actually rheumatic inflammatory changes in lungs are caused by capillarites and widespread or limited vasculites of preferential small branchings of pulmonary arteries. They make a basis of rheumatic pneumonia, thromboembolic processes, play a part in a pathogeny of infarctive, hypostatic pneumonia (see).
Rheumatic pulmonary vasculitis (a pan-vasculitis of small branchings of pulmonary arteries) it is found in 70 — the 80th years rather seldom. Developing against the background of or along with other manifestations acute, subacute or continuous retsidi-viruyushchego R., the rheumatic pulmonary vasculitis is clinically shown by short wind, cough, a pneumorrhagia, to-rye cannot be explained with pulmonary hypertensia owing to mitral heart disease or the expressed carditis. Essential diagnostic help is given by the repeated X-ray inspections finding dynamism inherent to a vasculitis, sometimes fleeting character of pathological symptoms.
The most often rheumatic pulmonary vasculitis arises at a long rheumatic carditis, against the background of mitral heart disease, especially in the presence of developments of stagnation in a small circle of blood circulation that complicates its recognition. Chronic vooialitelny defeat of vessels of lungs should be assumed in the presence of the asthma inadequate to expressiveness of valve defeat, the repeated pneumorrhagia amplifying during the periods of aggravations of rheumatic process, repeated heart attacks of the lungs which are complicated by infarctive pneumonia it is long recurrent infarktogenny pleurisy. Careful comparison of radiological data with clinical symptoms of existence and expressiveness of disturbance of blood circulation in lungs is important. According to E. S. Lepskaya (1967), radiological the vasculitis (unlike signs of stagnation at dekompensirovanny heart disease) is characterized by deeper and steady reorganization of the pulmonary drawing at the expense of an intersticial (perivascular) component. In nek-ry cases the symmetric multiple melkoochagovy shadows reminding a kartnna of miliary tuberculosis can be found. Also larger disseminated centers are described, to-rye as well as the sclerous processes arising after them, on roentgenograms have an appearance of snow flakes (a symptom of «blizzard»).
One of manifestations of the intersticial changes accompanying a rheumatic pulmonary vasculitis is the syndrome of capillary and alveolar blockade described by A. I. Nesterov (1973) and other researchers. Clinically it is characterized by the attacks of short wind, suffocation reminding cardiac asthma conceding not so much cordial, how many antirheumatic therapy.
Rheumatic pneumonia in a classical form at primary R. meets seldom. More often it is found in patients with nepreryv-but-retsidivpruyushchim returnable R.'s current in the presence of pleurisy and a pronounced carditis (pancarditis). Developing of pneumonia is clinically characteristic (see) against the background of an aggravation of rheumatic process, tendency to migration (sometimes plurality) focal changes, accurate effect of antirheumatic therapy in the absence of effect of massive doses of antibiotics.
The beginning of pneumonia is followed by fervescence, strengthening of an asthma, emergence of stethalgias, dry cough, substantial increase of indicators of activity of an inflammation, credits of antistreptococcal antibodies. Localization of process can be both unilateral, and bilateral. Classical physical symptoms of pneumonia can not be found or have the erased character. Radiological at acute rheumatic pneumonia allocate vascular and actually pneumonic phases. In the first changes of the pulmonary drawing at the expense of vascular and perivascular components, in the second — emergence on this background of quickly increasing and merging centers of consolidation are noted. At bilateral pneumonia the focal shadows merging in radical zones create a characteristic X-ray pattern («wings of a butterfly») against the background of the strengthened pulmonary drawing. Thus, rheumatic pneumonia is characterized by a number of the features allowing to differentiate it with other types of pneumonia at patients with rheumatism.
Rheumatic pleurisy as manifestation of a rheumatic serositis on frequency takes the second place after a pericardis. According to E. S. Lepskaya (1980), exudative forms of pleurisy are found only in 4 — 5% of sick R., an adhesive adhesive pleuritis (including a pleuropericarditis) at returnable R. radiological comes to light at 40% inspected. At primary Ruble. pleurisy (see) can be early manifestation of the acute period of the attack. In these cases tendency to bilateral defeat of serous covers of pleural cavities, preferential exudative character of an inflammation are characteristic. It is necessary to remember possibility of interlobar, phrenic pleurisy, in recognition to-rykh a crucial role belongs to a radiological method of a research. The same can be told also about adhesive pleurisy at returnable R., to-rye are clinically diagnosed not always. It is connected with difficulties in differentiation of actually rheumatic damage of a pleura from group of the so-called cardiac pleurisy including the infarctive and reactive serosites caused by nonspecific inflammatory processes in a congestive lung. Important differentsialno - diagnostic value has establishment of communication of variability of pleural defeats with dynamics of other clinical displays of rheumatism.
Damage of abdominal organs
Clinically at acute options of a current of R. is quite often noted passing increase in a liver and spleen. More resistant gepato-liye-naljny a syndrome (see) it can be found at a continuous retsidi-viruyushchem option of a disease. In this case special attention should be paid to differential diagnosis with a bacterial endocarditis. On the basis of studying of biopsy material by A. A. Likhachev (1970), at many sick R. in a liver changes from a stroma in segments are revealed and in portal system, dystrophy of hepatocytes. Along with it functional disturbances from a liver according to biochemical, fermentolo-gichesky researches are described. Rheumatic pancreatitis and a diabetes mellitus are mentioned in the literature devoted to questions of rheumatology, the passing disturbances of a functional condition of a stomach and intestines confirmed by special researches are described.
Rheumatic peritonitis represents clinical option of a rheumatic polyserositis. Meets usually at an acute current in a debut of the attack, it is almost exclusive at children's and youthful age (at 3 — 5% of patients). Serous or serofibrinous peritonitis is its cornerstone (see). The clinic is characterized by sudden emergence usually against the background of fever and other manifestations of acute R. of the diffusion or localized in this or that area of a stomach pains which are followed by nausea, sometimes vomiting, a delay or increase of a chair. Pains can be various on the expressiveness, migrating, skhvatkoobrazny and non-constant. Quite often they remind a pain syndrome at appendicitis (see), cholecystitis (see), paranephritis (see), stomach ulcer (see. Peptic ulcer ), what serves as the reason of diagnostic mistakes, and sometimes — an unjustified operative measure apropos acute abdomen (see). Objectively the small non-constant tension of an abdominal wall, not always accurate morbidity at a palpation is defined, it is rather rare — a symptom of Shchet-kina — Blyumberg. Involution of these symptoms in the next few days, usually without tendency to a recurrence is characteristic.
In diagnosis of rheumatic peritonitis its combination to these or those indisputable manifestations of R., unstable, often ephemeral nature of clinical manifestations, bright and bystry effect of antirheumatic therapy are important. It is essential as well characteristic dissociation between expressiveness of the general manifestations of R. and uncertainty, often erased character of local symptoms of peritonitis.
Damage of kidneys
Recognized is the provision that a basis of renal pathology at R. makes defeat of vascular system, first of all its capillary link. At the same time at different stages of development of rheumatic process depending on a degree of activity, character of a current, expressiveness of autoimmune, hemodynamic, toxi-infectious disorders various displays of renal pathology in various combinations can be found. So, with the acute course, the maximum activity of process rather often are found in patients short-term proteinuria (see), hamaturia (see). Diffusion focal less often glomerulonephritis (see) come to light usually at chronic, first of all a continuous and recurrent current of River.
Rheumatic damage of kidneys unlike a so-called congestive kidney is characterized by bigger firmness of an uric syndrome, preferential an erythrocyturia, decrease in concentration function of kidneys, positive dynamics of changes against the background of treatment by anti-inflammatory drugs. By data A. I. Strukova and A. I. Gritsyuk, in differential diagnosis it is necessary to consider also the thrombovasculites, heart attacks of kidneys found pathoanatomical in a final stage of R.
Damage of skin and hypodermic cellulose
carry a ring-shaped erythema To the characteristic rheumatic damages of skin designated by A. A. Kisel as «absolute symptoms of rheumatism» (see) and rheumatic small knots. The ring-shaped erythema (erythema annulare, erythema marginatum) is found usually at children's and youthful age, is more rare — at adults. Clinically it is shown light pink, sometimes with a bluish shade, sometimes hardly distinguishable ring-shaped enanthesis which are not followed by an itch or other subjective feelings. It is characteristic thin, places a disappearing rim of a ring with accurately outlined outside and more pale, indistinct inner edge. Diameter of rashes — from several millimeters to 7 — 8 cm. Sometimes primary elements of rash arise in the form of the light pink spots increasing in sizes with gradually turning pale center. The formed thin rings merge in the fancy polycyclic figures which are not towering over the level of skin easily disappearing during the pressing by a slide plate. Preferential localization — shoulders, a trunk, is more rare skin of shins, forearms, hips, necks, persons. The ring-shaped erythema can quickly arise and disappear also quickly, appears in any phase of development of the rheumatic attack. Arising against the background of widespread changes of a micro circulator bed inherent to active R., the ring-shaped erythema not always, however, is considered as manifestation of a skin vasculitis. Are suggested about the leading role of the vasomotor reactions arising in a sensibilized organism under the influence of vasoactive substances, disturbances of vegetative regulation. The ring-shaped erythema is so characteristic of R. that by right it is referred to the main diagnostic criteria of its active phase. At the same time it is not absolutely specific since it is described at sepsis, a glomerulonephritis, reactions medicinal intolerable ti and other states. At children the ring-shaped erythema is observed at a toksiko-allergic form of an adenoid disease. In addition to a typical ring-shaped erythema, at sick R. of children (is more rare than teenagers) also annulyaropodobny rash can be observed.
Rheumatic small knots (noduli rheumatici) as a distinguishing character of R. in domestic literature are for the first time described by A. A. Kisel in 1893. These are the roundish, dense, varying by the sizes from several millimeters to 1 — 2 cm painless educations. Skin over them is mobile, is not inflamed. Arising quickly, small knots are localized usually at places of an attachment of sinews, over bone surfaces and ledges, in knee, elbow, metacarpophalangeal joints, in anklebones, an akhillov (calcaneal) sinew, an occipital part of a tendinous helmet (galea aponeurotica). Having appeared, they can disappear within several days, but are exposed to involution only in 1 — 2 month without the defined residual changes more often. Most of researchers consider rheumatic small knots as analogs of ashoff-talalayevsky granulomas, distinctions with to-rymi are caused by features of local fabric reactivity. From the rhematoid small knots characteristic of a pseudorheumatism (see), rheumatic small knots differ in smaller size, plurality, lack of an accurate tendency to education fell-sadoobraznykh the structures revealed at microscopic examination of biopsy material.
As small knots refer characteristic manifestation of an active phase P. to the main diagnostic criteria of a disease. However they have no absolute diagnostic value (similar educations are described at other diseases) and are not a precursory diagnostic character. The diagnostic value of detection of rheumatic small knots considerably decreased also in connection with falloff of frequency of their manifestation for the last two — three decades.
Are much less characteristic in comparison with a ring-shaped erythema and rheumatic small knots and therefore the knotty erythema (erythema nodosum), a polymorphic exudative erythema (erythema exsudativum multiforme) and diseases arising at highly active forms observed at R. punctulate hemorrhagic rashes — peliosis rheumatica have no diagnostic value.
CRITERIA of DIAGNOSIS of RHEUMATISM
Despite achievements in development of modern diagnostic methods, establishment of the authentic diagnosis of R., especially its initial manifestations is frequent, makes not an easy problem for the doctor. Lack of specific clinical and laboratory diagnostic tests defines need of syndromic approach to establishment of the diagnosis of R. Sut of the last consists in reasonable idea that nosological specificity of a disease can be established at detection of a combination of nonspecific syndromes, characteristic of it. This principle made a basis of the diagnostic criteria of acute R. Ukazav offered by A. A. Kisel on the diagnostic importance of syndromes of a carditis, polyarthritis, a chorea, rheumatic small knots, a ring-shaped erythema, characteristic of this disease, he paid attention to importance of their combinations for reliable recognition of River. Later the same five syndromes are carried by the American cardiologist Jones (T. D. J ones, 1944) to the main («big») diagnostic criteria of «acute rheumatic fever». Besides, additional («small») clinical and laboratory criteria were marked out to them. The offered scheme was modified and approved by the American association of cardiologists in 1955 and 1965. The last option of review is presented in tab. 3.
The combination two «big» or one big and two «small» criteria indicates a high probability of R. only if symptoms of the previous streptococcal infection come to light (recently postponed scarlet fever, sowing from a nasopharynx of streptococci of group A, the raised credits of anti-streptolysin - 0 or other antistreptococcal antibodies). The scheme of diagnostic criteria of Jones in its modifications by right called in our country by the scheme of Kissel — Jones, gained universal distribution.
It is indisputable that the specified schemes of diagnostic criteria played and, undoubtedly, play a large role in development of more strict requirements to R.'s diagnosis, in R.'s isolation from group of similar syndromes and diseases. Fairly, however, and the fact that the recommended combinations of criteria not always guarantee reliability of diagnosis. Decrease several times for the last two — three decades of frequency of identification of rheumatic small knots, a ring-shaped eritekhma minimized the diagnostic value of these symptoms at R. Nedostatochno's recognition suitable there was this scheme of criteria and for early diagnosis of primary R., its long options that it is especially important for timely treatment and the prevention of serious consequences of a disease.
Developing the idea of syndromic diagnosis of primary rheumatism, A. I. Nesterov in 1973 suggested to allocate three main syndromes, the combination to-rykh allows to increase reliability of the diagnosis considerably. — to a klinikoepidemiologichesky syndrome it carried to the first anamnesticly accurately revealed streptococcal infection, aggravations a cut are in chronological communication with initial symptoms of R., existence of repeated nasopharyngeal infections. Ascertaining of such kliniko-epidemiological communication, according to most of researchers, makes one of paramount elements of the diagnosis of River. The following kliniko-immunological syndrome includes: an unmotivated delay of recovery of the general condition of the patient after the postponed nasopharyngeal infection, fatigue, heartbeat, arthralgias, non-constant subfebrile condition; detection in blood at the sick raised credits of antistreptococcal antibodies. Besides, even before emergence of the first clinical symptoms the changes of biochemical indicators accompanying an inflammation — a disproteinemia can be found (see. Proteinemia ), acceleration of ROE, increase in level of seromucoid and alpha 2 - globulins (see. Immunoglobulins ), emergence of S-reactive protein, etc. At last, the third necessary component of syndromic diagnosis of primary R. is a characteristic cardiovascular syndrome. The l revealed by means of the physicist ny, tool and graphic, radiological and other methods of a research, he confirms existence of a carditis and characteristic noncardiac manifestations of rheumatic process.
Comprehensive examination of patients with primary R., their katamnestichesky observation at Institute of rheumatology of the USSR Academy of Medical Sciences showed that use of the described syndromic method considerably increases reliability of diagnosis of primary R. including its long options.
The criteria of diagnosis of R. suitable for epidemiological researches have great practical value also developed by group of rheumatologists of the USSR (L. I. Benevolenskaya, N. A. Andreyev, etc., 1975). It is always necessary to mean, however, that any scheme will not replace active diagnostic and differential and diagnostic work of the doctor at least because recognition of any symptom or syndrome demands a certain clinical experience and sufficient level of medical qualification.
Idea of R. as about a general disease with preferential defeat of cardiovascular system, kliniko-morphological data on staging of its development taking into account an etiological role of a r-hemolitic streptococcus of group A proved creation of domestic system of three-stage treatment of R. Vydvinutoye in 60 — the 70th years A. I. Nesterov the kliniko-immunological direction in R.'s studying made an essential contribution to its further development. In a modern look this system includes: treatment of an active phase of a disease in a hospital, continuation of treatment after an extract from a hospital in a rheumatological office of policlinic for adults, or a cardio-rheumatological office of children's policlinic, or in suburban sanatorium with connection of rehabilitation actions, the subsequent long-term dispensary observation and preventive out-patient treatment. More than twenty years' experience of fight against rheumatism in the USSR confirmed high performance of three-stage treatment and systematic long-term observation of patients in the conditions of policlinic. Success of treatment depends on that, its program depending on a degree of activity, option of a course of rheumatic process, expressiveness of a carditis, weight of valve and muscular damage of heart, existence of streptococcal contamination is how individualized. In general such program consists of antimicrobic and antiinflammatory therapy, actions directed to recovery of an immunological homeostasis, the organization of the adequate mode, balanced food, to adaptation to exercise and labor stress, timely operational treatment of heart disease, focal infection.
Patients with an active phase of a disease are subject to treatment in a hospital, as a rule. With the maximum or moderate activity of rheumatic process preferential bed rest within 2 — 3 and more weeks is shown. Its duration is defined also by expressiveness of a carditis, disturbance of compensation of cordial activity and other complications. Expediency of rather early expansion of a physical activity with rational use of the individualized complexes of remedial gymnastics is established, however. Experience shows also that patients with an active phase P. do not need a rigid special diet. It is necessary at the same time take into account that in the feverish period food shall be vitamin-rich, easily an usvoyaema, is rather nutritious, but not a vysokokaloriyna. The thesis about pathogenetic value of excess of carbohydrates remains at least debatable. The diet of the patient with rheumatism shall include enough complete proteins (not less than 1 g/kg of body weight) and lipids.
Causal treatment of R. is carried out by the penicillin having bactericidal effect on hemolitic streptococci, including groups A of all types. One of conditions of efficiency of antistreptococcal treatment is its sufficient duration. To this task there corresponds not less than two-week purpose of injections of penicillin in the period of an active phase with the subsequent transition to use of the prolonged drug of Bicillinum-5. The recommended doses: 1 200 000 — 1 500 000 PIECES of potassium or sodium salt of penicillin a day during 2 weeks, then on 1 500 000 PIECES of Bicillinum-5 once in 4 weeks. At intolerance of penicillin erythromycin on 0,25 g 4 times a day during the same term can be recommended. Use of streptocides and tetracycline drugs is not justified because of possibility of stability of strains of a streptococcus to them.
For the purpose of the prevention of streptococcal superinfection it is recommended to place patients in the low-local, regularly aired chambers, to make their uv radiation, to provide strict observance by patients of measures of personal hygiene. For identification chronic tonsillitis (see) and careful conservative, and if necessary — its operational treatment, all sick R. are inspected and observed by the otorhinolaryngologist. As a rule, the tonsilectomy is shown only after an extract of the patient from a hospital at normalization or considerable decrease in indicators of activity of rheumatic process.
Antiinflammatory and immunodepressive therapy — one of the most important links of actually pathogenetic therapy of rheumatism. To to anti-inflammatory drugs (see), applied to treatment of an active phase of a disease, the glucocorticosteroid hormones derivative salicylic to - you (pyrazolon and indole), derivatives of phenylacetic acid, etc. belong. To to antirheumatic means (see) preferential immunodepressive effect with secondary antiinflammatory effect derivatives of quinoline (delagil, plaquenil) belong.
Differentiation of options of a current, allocation of degrees of activity of R. assumes also the differentiated approach to holding medical actions in the corresponding clinical groups. The scheme of treatment of R. at children generally same, only doses of medical drugs change. The pronounced chorea demands purpose of complex hormonal and medicamentous therapy as any of separately the appointed pharmaceuticals does not render due therapeutic effect.
Early the begun vigorous antiinflammatory therapy is capable to warn or to considerably limit development of rough fibrous deformations of valves of heart. It is especially necessary for treatment of the acute and subacute options of disease which are most often revealed at primary by R. Metod of the choice for treatment of patients of this category use of glucocorticoid hormones (Prednisolonum on 20 — 30 mg a day) in combination with non-steroidal anti-inflammatory drugs is. It is shown that such treatment at the specified options of disease allows without essential collateral manifestations for short term to make the stopping impact on active rheumatic process.
Other tactics of medicinal therapy is used at the chronic options of disease (first of all at long and latent) developing usually at returnable rheumatism is frequent in the presence of already far come changes in the valve device of heart and in a myocardium. The reduced nature of inflammatory reactions, the expressed dystrophic changes, disturbances of protein, energy, water and electrolytic metabolism define insufficient efficiency at these sick glucocorticosteroid drugs. Long-term clinical experience and specially conducted researches testify to much bigger expediency of appointment in these cases of derivatives of quinoline (delagil, plaquenil) for the term of not less than a year in combination with the monthly injections of Bicillinum-5 and means exerting beneficial effect on the broken metabolic processes. Non-steroidal anti-inflammatory drugs are appointed also to a long term in the doses adequate to a degree of activity of rheumatic process taking into account expressiveness of its aggravations. For the purpose of impact on a current of a chronic vasculitis, hypercoagulation, the increased aggregation of thrombocytes at long, continuous retsidi-viruyushchikh options P. purpose of such drugs as andekalin, komplamin, curantyl is shown. Methods of treatment continuous retsidi-viruyushchego option of a current of R. at height of the exacerbation of a disease which is followed by pronounced inflammatory reaction shall be especially thought over and individualized. In this period appoint glucocorticosteroids and high doses of non-steroidal anti-inflammatory drugs.
The second link of system of step-by-step treatment of R. provides stay of the patient, especially the child and the teenager, in local rheumatological or adults in cardiological sanatorium and further treatment in polyclinic conditions. A main objective — continuation of antirheumatic therapy by nonsteroid antiinflammatory and quinolinic drugs, systematic introduction of Bicillinum-5, use of a medical pshnastika and the tempering procedures for the purpose of recovery of the violated protective functions.
Dispensary observation and preventive treatment of sick Ruble enters a problem of the third link. At the same time treat problems of medical examination: implementation of the medical actions directed to final elimination of active rheumatic process; treatment of disturbances of blood circulation at patients with heart diseases, the decision together with the cardiosurgeon of questions of surgical correction of defects, carrying out at indications of a tonsilectomy; solution of a question of a possibility of purpose of a klimatobalneoterapiya; solution of questions of rehabilitation, working capacity, employment; implementation of secondary prevention of a recurrence of a disease. In tab. 4 approximate skhekhm (options) of medicinal therapy of patients with rheumatism at consecutive treatment are given them in a hospital, policlinic, sanatorium depending on the course of rheumatic process.
Physiotherapy exercises. LFK is an important component of complex treatment of R. at all stages: a hospital, policlinic, sanatorium, including independent classes at home in special recommendations. The important place is taken by prevention of aggravations by means of a systematic training physical exercises and hardening (see). LFK at defeat of cardiovascular system promotes compensation of heart failure by impact on noncardiac factors, improvement of exchange processes in a myocardium. The remedial gymnastics at the bed rest ordered to the patient has unloading character, putting the task to facilitate cardiac performance. In a prone position simple exercises at slow speed are applied to separate muscular groups in combination with not forced breathing exercises lasting occupations of 5 — 7 min. 2 — 3 times a day. At a bed rest the remedial gymnastics is carried out in a prone position and reclining, and at expansion of the mode — lying and sitting. Slow rate of exercises alternates with an average, exercises for hands and legs, and then a trunk in combination with breathing exercises are used, duration of the procedure reaches 15 min. At-lupostelnom after the mode when LFK puts the task and adaptation of cardiovascular system to the increasing dosed loading, are applied morning hygienic and remedial gymnastics, to-rye include in a standing position and during the walking of exercise for all muscular groups with elements of effort (exercise on resistance, with burdening), and also exercises for development of a correct posture using plays of small and medium mobility and breathing exercises; duration of the procedure is 20 min. At the LFK ward mode it is carried out in an office with use of various initial positions, gymnastic apparatus, with wide use of plays of average mobility — lasting 25 — 30 min., and obligatory carrying out morning hygienic exercises. At a general regime many means of LFK are used: morning hygienic and remedial gymnastics, the dosed walking at walks, independent classes in tasks. In the procedure of remedial gymnastics, lasting up to 35 min., include bystry walking, run, jumpings up, various games. At all stages of treatment massage is shown.
LFK at heart diseases — see. acquired, physiotherapy exercises .
At arthritis of LFK it is shown in a phase of subsiding of process. It is reasonable to begin a training with not affected joints, gradually passing to exercises on sore joints. In a prone position apply elementary exercises in the direct directions of movements, carefully, without causing or without strengthening the available pain. During the subsiding of the acute phenomena the volume of means of physiotherapy exercises increases: gymnastic exercises with load of all joints, with broad use of various shells and special devices are used.
LFK at a chorea is shown during the subsiding of the acute phenomena. At the beginning of a course of treatment the procedure of remedial gymnastics is carried out lying, exercises shall be simple, be carried out at quiet speed by means of the instructor. In process of development of exercises and reduction of degree of hyperkinesias of the movement become complicated, coordination exercises, balance join. Duration of the procedure from 5 — 10 min. to 20 min. twice a day, besides,
Early diagnosis, timely appointed adequate therapy connect board games, drawing, a molding, etc. == the FORECAST ==, the correct use of all links of stage treatment promote suppression of activity of rheumatic process, the prevention of formation of defect of heart - considerably to reduction of palindromias. R.'s forecast is defined by hl. obr. result of damage of heart, most often valve endocarditis (valvulitis). Therefore forecasting of an outcome of primary rheumatic valvulitis has paramount value for R.'s forecast in general. H. N. Kuzmina, A. V. Trufanova, T. G. Eye (1978) the predictive tables for the children's contingent of sick R. allowing to predict an outcome of a disease by results of klinikoinstrumentalny inspection are developed by means of the COMPUTER; also assessment of various schemes of treatment depending on the predicted outcome of a valvulitis is carried out; comparison of the obtained data allows to choose the complex of treatment providing an optimum outcome for the specific patient.
Identification of a role of a streptococcal infection in R.'s emergence and its recurrence allows to perform primary prevention of a disease. It consists of the measures directed to a systematic and reasonable hardening of an organism, development of physical culture and sport, holding the broad sanitary and hygienic actions reducing a possibility of streptococcal infection of the population, first of all children's collectives. Measures for timely recognition and effective treatment of both the chronic, and acute proceeding streptococcal infection are important. During the developing of the acute nasopharyngeal infections caused by a streptococcus except isolation of patients and observance of a bed rest, perhaps early purpose of the corresponding treatment is of great importance.
Secondary prevention is directed to the prevention of a recurrence and progressing of a disease at the persons who already transferred to R. Kroma of the actions relating to primary prevention it includes methods of the year-round and seasonal bitsillinomedikamentozny prevention of a recurrence performed by vra-chami-rheumatologists of ambulatornoprofilaktichesky institutions or district doctors. The year-round method of prevention of a recurrence of R. used in the Soviet Union is carried out by regular injections of Bicillinum-5 in a dose of 600 000 PIECES of 1 times in 3 weeks to children of preschool age, 8 years — 1 200 000 PIECES of 1 times in 4 weeks, to teenagers and adults — 1 500 000 PIECES of 1 times in 4 weeks are more senior.
During the spring and autumn periods during 4 weeks acetylsalicylic acid the adult in a dose of 2,0 g, to children of 1,0 — 1,5 g a day or other non-steroidal anti-inflammatory drug is in addition appointed. After 3 years' performing year-round bitsillino-prevention in the absence of a recurrence the next 2 years seasonal prevention is appointed. It is carried out in the spring and in the fall within 6 — 8 weeks by Bicillinum-5 in the dose of 1 500 000 PIECES entered monthly in combination with acetylsalicylic acid in a dose of 2,0 g a day. Patients with chronic options of a current of R. along with the listed above preventive actions need to pay especially much attention to the measures promoting recovery of the broken reactivity, compensation of functions of cardiovascular system. The wide experience of rational use of resort factors in stage treatment of River is accumulated.
Among rehabilitation actions the important place belongs to operational treatment of heart diseases, results to-rogo substantially depend on the preoperative preparation which is carried out by rheumatologists and postoperative observation of patients.
See also Collagenic diseases .
Table 1. CLINICAL LABORATORY CHARACTERISTIC of DEGREES OF ACTIVITY of RHEUMATIC PROCESS
Table 2. OPTIONS of the COURSE of RHEUMATISM, THEIR TEMPORARY CHARACTERISTIC AND FEATURES of the MAIN CLINICAL MANIFESTATIONS
Table 3. The BIG AND SMALL CRITERIA of RHEUMATISM (reconsidered, 1965)
Table 4. APPROXIMATE SCHEMES (OPTIONS) of MEDICINAL THERAPY of PATIENTS with RHEUMATISM AT THEIR CONSECUTIVE TREATMENT IN the HOSPITAL, POLICLINIC, SANATORIUM DEPENDING ON the COURSE of RHEUMATIC PROCESS
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A. I. Nesterov, V. A. Nasonova, I. A. Bronzov, A. V. Dolgopolova, H. N. Kuzmina; V. V. Mikheyev (not BP.), G. S. Fedorova (to lay down. physical.), M. A. Tsivilko (psikhiat.), A. B. Shekhter (stalemate. An.).