PSEUDORHEUMATISM

From Big Medical Encyclopedia

PSEUDORHEUMATISM (Greek rheuma, rheumatos, flow, stream + eidos look; arthritis[es]; synonym: infectious nonspecific polyarthritis, rheumatoid arthritis) — a general inflammatory disease of connecting fabric with preferential damage of joints as the chronic progressing erosive and destructive polyarthritis.

River and. — the most frequent disease among chronic polyarthritises (see). More often (by 2 — 3 times) women are ill, however it is ralzlichy is not so expressed at children's and advanced age.

The etiology

the Aetiology is not established. The opinion on possible participation in an origin of a disease of three factors is conventional: infections, disturbances from immune system and genetic predisposition.

Attempts to find the infectious agent, being the activator P. and., remain still unsuccessful. In different spans the attention was drawn to a streptococcus, diphtheroids, clostridiums, mycoplasmas, various viruses. However direct proofs of participation of these infectious agents in R.'s development and. it is not received. Apparently, even if such activator exists, the mechanism of its action does not come down to direct damage of tissues of joint and is much more difficult. The assumption that to R.'s etiology is made and. various microorganisms possessing nonspecific adjuvant impact on immune system, disturbances from outside a cut can be related (on condition of genetic predisposition) define further disease. Also the possibility of influence of viruses on the genetic device of cells of tissues of joint with the subsequent disturbance of synthesis of a number of proteins, in particular superficial receptors is not excluded owing to what it is lost immunol. tolerance. In the latter case it is not possible to find a virus by the standard methods. Option of the last hypothesis is the assumption of possible primary impact of a viral infection on immune system, first of all on its regulating mechanisms.

At R. and. disturbances are found practically from all links of immune system: humoral (existence of autoantibodies, first of all rhematoid factors (see), and also cell-bound immune complexes), cellular (ratio distortion T-and V-lymphocytes, the proliferative answer to mitogens, etc.), the englobing cells (decrease in hemotaksichesky and phagocytal functions). The essential proof of value of immune system in R.'s development and. the expressed efficiency of removal of lymphocytes at a drainage is chest limf, a channel. Nevertheless specific value revealed immunol. disturbances in R.'s development and. remains not clear. As the frequency of their detection is changeable, and expressiveness depends on activity of a disease, it is more than data for the fact that they are secondary. Primary defect of immunity at R. and. it is not found yet; there are bases to assume communication it with the regulating mechanisms of immune system.

Opening in the 70th of the regulating subpopulations of lymphocytes (T lymphocytes of suppressors and helper) led to creation of a hypothesis of possible decrease at R. and. activities of T lymphocytes of the suppressors which are slowing down normal functions of V-lymphocytes. Decrease in suppressor function of T lymphocytes at sick R. and. it was confirmed in a number of researches. The disproportion among the regulating subpopulations of T lymphocytes is revealed in a synovial membrane of joints at sick R. by ampere-second the help of monoclones. One more confirmation of this hypothesis was successful use at R. and. the pharmaceuticals capable to strengthen function of T lymphocytes (levamisole, timopoetin).

Existence of genetic predisposition at R. and. it is established for a long time, on the basis of such known factors as the increased frequency of a disease among relatives of patients of the first and second degree of relationship, the big frequency of a simultaneous disease of R. and. at monozygotic twins, than at dizygotic. At R. and. certain features are found in distribution of histocompatibility antigens. So, according to P. Stastny and soavt. (1978, 1979), HLA Dw 4 is found in 52% of sick R. and. and only in 13% in population; more often than in population, according to Batchelor, Morris (J. R. Batchelor, P. J. Morris, 1978), also HLA DRw 4, and according to L. I. Benevolenskaya et al. (1981), V 18 meet, Bw 35 and V 27, is more rare — HLA DRw 2 [G. S. Panayi et al. (1978)].

Creation of the harmonious theory of an etiology of R. and., combining in itself all three brought groups of the facts, is a problem of future researches.

A pathogeny

mechanisms of the main manifestation of R. are Most studied and. — synovitis (see). Hypothetical etiol. the factor damages a synovial membrane (a synovial membrane, T.), therefore the response immune response which is followed by formation of aggregated develops immunoglobulins (see). The changed IgG is distinguished by immune system as alien antigen, and plasmocytes of a synovial membrane, limf, nodes, spleens begin to develop to it autoantibodies (rhematoid factors). Interaction of the aggregated IgG with rhematoid factors and complement (see) leads to formation of cell-bound immune complexes that, in turn, promotes aggregation of thrombocytes (to formation of microblood clots, release of vasoactive amines), stimulates allocation of cytotoxic factors from lymphocytes (see. Mediators of cellular immunity ), the cut activates system of a complement, separate components have anaphylactogenic, cytotoxic and hemotaksichesky properties. The last promotes inflow of a large number of neutrophilic leukocytes to a joint cavity. Phagocytosis of cell-bound immune complexes neutrophilic leukocytes leads to their destruction and release of lizo-somalny enzymes — hydrolases (see), capable to activate a number of the mediators of an inflammation (a histamine, serotonin, kinina, prostaglandins, factors of a complement, etc.) playing an important role in development of inflammatory, and then destructive and proliferative changes of a synovial membrane. Damage of tissues of joint and accumulation of the aggregated IgG stimulates an immune response, infiltration of these fabrics plasmocytes and sensibilized lymphocytes extends and goes deep, to-rye have the direct damaging effect on tissues of joints, and also allocate the mediators promoting activation of neutrophilic leukocytes. A row so is created. naz. vicious circles (see. Circulus vitiosus ), therefore patol. process becomes chronic.

Pathogeny of the majority of extraarticular (system) manifestations of R. and. it is connected with development vasculitis (see), and also, perhaps, with direct damage of fabrics by sensibilized lymphocytes or autoantibodies.

Experimental data

the Pilot or natural model which is adequately imitating R. and., it is not found yet what, certainly, complicates studying of its etiology and pathogeny. Nevertheless in an experiment it is possible to receive subacute or hron. the inflammation of joints reminding to some extent the changes which are observed at R. and.

The greatest distribution, hl. obr. for the purpose of experimental studying of antirheumatic means, got the so-called adjuvant arthritis caused in rats at intradermal introduction of a full Freund's adjuvant. Approximately in 2 weeks polyarthritis with a periarthritis develops after that, to-ry proceeds within several weeks and then independently abates. Idiosyncrasy is development of calcification of circumarticular fabrics that brings to an anchylosis of joints. It is established that have «artritogenny» properties peptide of an oglikana, consisting of a chain of the alternating molecules N-atsetilmuramovoy to - you, about a cut are connected tetrapeptide of a certain structure. Peptide of an oglikana of the specified structure are structural components of inner membranes of bacteria of the most various types.

There is also a model hron. arthritis, the developed W. Y. Cromatrie et al. At intraperitoneal introduction to rats of a sterile aqueous slurry of fragments of a cell wall of various bacteria (usually streptococci) develops hron. the polyarthritis which is characterized by erosive process. It is supposed that in this case, as well as at adjuvant arthritis, the major pathogenetic importance have peptide of an oglikana of a cell wall.

Natural model P. and. in a certain measure it is possible to consider erizipeloid — inf. the disease of pigs caused by Erysipelothrix rhusiopathiae and which is characterized acute or hron. arthritis. At the same time the thickening of a synovial membrane, development in it vorsin and considerable lymphoid infiltration, i.e. changes reminding a rhematoid synovitis is noted.

Pathological anatomy

For R. and. involvement in patol is characteristic. process of many bodies and systems, but preferential are surprised joints. At this disease

the immune inflammation which is expressed in the form of hypersensitivity of the immediate and slowed-down type is the cornerstone of fabric damages. Inclusion of factors of humoral allergies (see) is confirmed by signs of sharp strengthening of vascular permeability, presence in sites of defeat of immunoglobulins and a complement, polymorphonuclear leukocytes, plasmablasts, proliferative and destructive character of a vasculitis. Morfol. expression of cellular hypersensitivity is existence of a large number of blast forms of T lymphocytes (see. Immunocompetent cells ) and macrophages (see) with signs of intensively proceeding phagocytosis (see) in the struck fabrics. In development of fabric and organ damages at R. and. the leading role is played by microcirculator disturbances and lysosomic enzymes.

Fig. 1. The diffraction pattern of a synovial membrane of a joint at a pseudorheumatism received by means of the scanning microscope: fleecy growths are visible; X 35 000.

In joints at developed a wedge, a picture of a disease fleecy growth of the synovial membrane (fig. 1), regarding cases crawling in the form of a pannus over the surface of a joint cartilage is found. At the same time in a joint cartilage erosion, cracks, cracks are defined; its remains dry and granular by sight. In joint cavities the increased amount of viscous muddy synovial fluid is found, the thickening and a sclerosis of the joint capsule is constantly noted, there are fibrous commissures. The full obliteration of a joint cavity commissures leads to development of fibrous anchylosis (see). Proliferation of ossiform fabric is followed by development osteophytes (see) and bone ankiloz.

Fig. 2. Microdrug of a synovial membrane of a joint at a pseudorheumatism: proliferation of sinoviotsit (1) with their palisadoobrazny arrangement in relation to fibrinopodobny imposings (2) on a surface of a synovial membrane; coloring hematoxylin-eosine; x 400.
Fig. 3. The diffraction pattern of sinoviotsit of type A of a synovial membrane of a joint at a pseudorheumatism: hyperplasia of lysosomes (1), mitochondrions (2), granular cytoplasmic reticulum (3) and filopodiums (4); X 11 600.
Fig. 4. Microdrug of a synovial membrane of a joint at a pseudorheumatism: diffusion lymphoid plazmotsitarnaya infiltration and proliferation of sinoviotsit (are specified by shooters); coloring hematoxylin-eosine; X 200.

In gistol. allocate to a picture of a synovitis the following changes: 1) defeat of vessels of a microcirculator bed; 2) hyperplasia and hypertrophy of synovial cells; 3) inflammatory cellular infiltration; 4) growth of granulyatsionny fabric, sclerosis, lipomatoz synovial own plate. The permeability of walls of vessels a microcirculator-nogo of a bed which is followed by an exit of fibrinous exudate and formation of the centers of fibrinoid in synovial own plate and fibrinopodobny imposings on a surface of a synovial membrane increases. Immunomorfologicheski in the centers of fibrinoid and in fibrinopodobny imposings fibrin, immunoglobulins (G, And, M) and C3 fraction of a complement come to light. At an aggravation of an inflammation the productive or productive and destructive vasculitis with fixing in a vascular wall of immunoglobulins and a complement is constantly noted. In deep layers synovial membrane (see) rhematoid small knots — the small sites of a fibrinoid necrosis surrounded with macrophages and lymphocytes occasionally are defined. In 80% of cases the hyperplasia and a hypertrophy of sinoviotsit meets their characteristic palisadoobrazny arrangement (fig. 2). By means of electronic microscopic examination it is revealed that the hypertrophy of sinoviotsit occurs at the expense of a hyperplasia of their organellas, especially granular cytoplasmic reticulum, the policy, Golgi's device and lysosomes (fig. 3). Sinoviotsita of all three types participate in a hyperplasia of a fleecy layer of a synovial membrane (And, In and C). At early stages of an aggravation of an inflammation cellular infiltration in a synovial membrane scanty, is presented by lymphocytes, macrophages and polymorphonuclear leukocytes. Further infiltration (fig. 4), sometimes with formation of lymphoid follicles is noted lymphoid plazmotsitarnaya. In parallel at immunofluorescent studying of T - and V-lymphocytes of a synovial membrane at R. and. A. D. Bankhurst et al. (1976) revealed dominance of T lymphocytes. In a synovial membrane considerable proliferation of fibroblasts is noted. In synovial own plate and a fibrous layer growth of granulyatsionny fabric from the outcome is observed in sclerosis (see) and lipomatoz (see).

Destruction of a joint cartilage happens hl. obr. at the expense of a pannus. The cartilage under a pannus is thinned, has deep cracks, the eosinophilia of the main substance and reduction of quantity of chondrocytes with disturbance of zonality of their arrangement are noted. In the absence of a pannus of change in a joint cartilage take usually superficial zone, in a cut small uzura, fibrillation of a matrix, dystrophic changes of chondrocytes, and also sharp reduction of maintenance of glikozaminoglikan come to light. In a joint cartilage at the same time cell-bound immune complexes are found. In destruction of a joint cartilage the significant role is played also by disturbance of exchange of synovial fluid and a marrowy pannus.

Fig. 5. Microdrug of a rhematoid node at a pseudorheumatism: necrotic masses (1) is surrounded with shaft from palisadoobrazno the located histiocytes (2), to the Crimea granulyatsionny and fibrous fabric (3) adjoins; coloring hematoxylin-eosine; X 200.

In patol. process at R. and. practically all internals can be involved. Most often kidneys are surprised, in to-rykh comes to light amyloidosis (see), more rare glomerulonephritis (see) and pyelonephritis (see). The amyloidosis of a liver is sometimes observed, went.-kish. path, heart, spleen. Damage of heart and lungs is most often connected with polyserositis (see) or its effects in the form of fibrous commissures in cavities. In nek-ry cases it is found valve endocarditis (see) with formation of heart disease, myocarditis (see) and cardiosclerosis (see), coronaritisis (see) and a coronarosclerosis, chronic pneumonia (see) and pneumosclerosis (see), caused by the basic patol. process. In heart and lungs rhematoid small knots can meet. Changes in c. N of page, as well as in other bodies, are connected with the vasculitis which is followed by death of nervous cells, demyelination and glybchaty disintegration of axial cylinders of axons. In skeletal muscles at high general activity of a disease the picture local is noted miositis (see). In isolated cases in hypodermic cellulose rhematoid nodes come to light, to-rye on a section have an appearance of the crumbling gray-yellow masses surrounded with the fibrous capsule. At microscopic examination in them unstructured necrotic masses is defined, to the Crimea the shaft from palisadoobrazno the located histiocytes (fig. 5) adjoins.

A clinical picture

According to the working classification accepted in the USSR allocate R. and., the shown mono - oligo-or polyarthritis, R. of ampere-second system (extraarticular) manifestations. To special forms P. and. carry Felti's syndrome (see. Felti syndrome ), Kaplan's syndrome (see. Kaplana syndrome ), Shegren's syndrome (see. Shegrena syndrome ). Distinguish also seropositive R. and. (in the presence of rhematoid factors in blood serum) and seronegative R. and. (at their absence). Existence of rhematoid factors (see. Rhematoid factor ), especially in a high caption, has diagnostic and predictive value, as formed the basis for introduction of this immunol. characteristics in classification.

So, at detection of with firmness high credits of rhematoid factors of R. and. it is characterized by the persistent course, bystry increase of erosive and destructive process in joints and development of various manifestations of a rhematoid vasculitis. Seronegative R. and., on the contrary, proceeds is more good-quality and it is characterized by defeat of smaller number of joints; extraarticular signs, as a rule, are not found. Besides, lack of rhematoid factors demands an exception of other options hron. arthritises (see. Arthritises ). In classification three degrees of activity depending on expressiveness of arthritis, number of the affected joints, extraarticular manifestations and changes of indicators of activity of an inflammation, and also option of a current — quickly progressing and slowly progressing are also allocated. Are also reflected in classification rentgenol. stages of process according to Staynbrokker's scheme (see below) and three degrees of functional insufficiency of joints.

Fig. 1. Brush of the patient pseudorheumatism: fingers have the spindle-shaped form caused by arthritis of proximal interphalangeal joints. Fig. 2. Brush of the patient pseudorheumatism: deformation of fingers as «a neck of a swan». Fig. 3. Forearm and distal part of a shoulder of the patient pseudorheumatism: shooters specified hypodermic rhematoid nodes. Fig. 4. A brush of the patient with a pseudorheumatism at digital arteritis: shooters specified dot necroses of skin of fingers. Fig. 5. An extensive ulcer of an outer surface of the lower third of a shin of the patient with a pseudorheumatism with a vasculitis. Fig. 6. Patient with a juvenile pseudorheumatism: rhematoid rash on a front surface of a thorax and stomach (it is especially expressed in the field of costal arches) against the background of a dermahemia.

The onset of the illness can be acute and subacute, but the most typically gradual slow development, at Krom as if persistent pains, feeling of constraint and a swelling of joints gradually develop. In some cases patients note communication of these phenomena with various (generally acute respiratory) infections, injuries, a nerve strain, pregnancy and childbirth. Usually at first the small number of joints is surprised, proximal interphalangeal, metacarpophalangeal and metatarsophalangeal joints, and also radiocarpal, intercarpal and knee are more often. Symmetric damage of joints is characteristic. At defeat of proximal interphalangeal joints fingers of a hand get a spindle-shaped form (tsvetn. fig. 1). Approximately in 10% of cases the disease begins and is long proceeds in the form of mono - or an oligoarthritis of large joints. Pains usually moderate also arise only at the movements. The characteristic feeling of constraint in joints is more expressed in the mornings.

The option P. seldom occurs at adults and., characterized by the acute beginning, high tvkhmpera-Tura, and also existence of various extraarticular manifestations that is more typical for juvenile R. and. High temperature reaction from oznoba — the first and most expressed symptom of this option of a disease. On the first place in a wedge, a picture also symptoms act intoxications (see), signs of systemacity of process (serosites, carditis, increase limf, nodes, liver, spleen), leukocytosis, acceleration of ROE. The phenomena of arthritis can be absent in the beginning in general (only polyarthralgias are noted) or to be moderately expressed. Only several weeks later or even months the resistant joint syndrome is shown. In dalneyshvkhm the tendency to aggressive disease with preservation during the periods of exacerbations of fever quite often remains.

Seldom initial manifestations of R. and. have character of recurrent arthritis, in some cases indistinguishable from palindromny rheumatism (see). Arthritis of one or several joints develops sharply or subacutely and without treatment completely passes through a small time term. The repeating attacks of arthritis can precede development of typical resistant for a number of years polyarthritis (see).

In typical cases at survey the swelling of the affected joints, their morbidity at a palpation and compression are noted (a symptom of compression of brushes, feet, etc.), a hyperthermia of skin in joints, color the cut is, as a rule, not changed. Already in the onset of the illness one of the most characteristic symptoms of a disease — symmetry of damage of joints is noted.

Quite often the inflammation of vaginas of sinews and sinews develops. Damage of the vagina of sinews of razgibatel of fingers located in radiocarpal and intercarpal joints is typical, a cut can lead to a prelum of a median nerve that is followed by paresthesias in the III—IV fingers and an atrophy of muscles of an eminence of a thumb (a syndrome of a carpal tunnel). Owing to damage of sinews of the muscles which are attached to a humeral bone humeroscapular can be observed periarthritis (see). A floor rested the expressed inflammation of a bag an eponchaty muscle, accumulation of exudate in it is followed by its stretching and can lead to emergence in a popliteal space of the palpated education — so-called cysts of Baker, or a cyst of a semimembranous synovial bag (see. Popliteal space ).

Slow, steady progressing of a disease is in most cases noted. Expressiveness a wedge, manifestations usually increases after various inf. diseases, injuries, the increased insolation, overcooling, childbirth, and it is frequent without the visible reasons. At early stages of a disease spontaneous remissions can be noted, short are more often. Gradually other joints are involved in process, destruction of a cartilage and a subchondral bone plate develops, function of joints is broken. At R. and. any joints, including temporal and mandibular, grudinoklyuchichny, acromial and clavicular, joints of acoustical stones, cartilages of a throat, and also vertebral joints of cervical department of a backbone can be surprised. However arthritis of distal interphalangeal joints, joints of chest and lumbar departments of a backbone and lumbosacral joints is not characteristic of a pseudorheumatism.

Resistant synovitis (see), and also the inflammation of the joint capsule, the sinews and their vaginas and bags which are reported with joints lead to development of deformations of joints, contractures, incomplete dislocations, ankiloz over time. Nek-ry types of deformations of joints are typical almost only for R. and. Treat deformation of fingers of a brush in the form of «a neck of a swan» them (tsvetn. fig. 2) — a flexion contracture of metacarpophalangeal joints with overextension in proximal and bending in distal interphalangeal joints, deformation of a finger in the form of «a button loop» — a flexion contracture proximal and overextension distal interphalanx joints (position of a finger during the fastening of a button), an incomplete dislocation of metacarpophalangeal joints with a deviation of fingers in the elbow party (a brush in the form of a fin of a walrus). These three types of deformations make a concept of a so-called rhematoid brush.

Molotochkovidny deformation of fingers of foot, incomplete dislocations of metatarsophalangeal joints with development flat-footedness (see), hallux valgus (see), and at damage of an ankle joint as well valgus deviation of foot, carry the name of rhematoid foot. Typically also development of flexion contractures of elbow and knee joints.

One patient can have various nature of damage of different joints: in one joints inflammatory changes, a hyperthermia, pain, in others — a thickening of periartikulyarny fabrics, flexion contractures prevail. But more often these changes are combined in one joint. Permanent damage of joints is followed by a degrowth of adjacent muscles. The muscular atrophy develops earlier and is most expressed on a back of the hand at arthritis of small joints (see. Atrophy muscular ).

Extraarticular manifestations of R. and. and damages of internals seldom act into the forefront. Their frequency increases in process of progressing of a disease, especially at polyarticulary and seropositive forms. Others hypodermic rhematoid small knots, a polyneuropathy and a rhematoid vasculitis meet more often.

Hypodermic rhematoid small knots (usually painless, moderately dense, the small sizes) are located in the field of an elbow shoot and a proximal part of an ulna more often (tsvetn. fig. 3), and also in joints of fingers of brushes, big spits, on an aponeurosis of a tendinous helmet of a skull, etc.

Polinevropatiya — manifestation of a vasculitis of the vessels feeding peripheral nerves (vasa nervorum). Defeat of distal departments of nervous trunks, most often the general fibular nerve, with development of disturbances of sensitivity is characteristic. Patients are disturbed by numbness, burning, a chill in distal departments of extremities. At a palpation morbidity not only the affected joints, but also surrounding fabrics is noted. There are motive frustration much less often. As a rule, the polyneuropathy is noted at patients with a seropositive form P. and. along with existence of hypodermic small knots.

Rhematoid vasculitis (hl are surprised. obr. vessels of small and average caliber) is the cornerstone of some other, more rare than the found extraarticular manifestations: the dot sites of a necrosis located most often in the field of a bed of a nail — so-called digital arteritis (tsvetn. fig. 4), the painless ulcers which are located usually on the lower third of a shin (tsvetn. fig. 5), an episcleritis, microheart attacks or hemorrhages in the pool of pulmonary, brain, coronary or mesenteric vessels.

Fig. 6. The roentgenogram of a thorax at a pseudorheumatism (a direct projection): shooters specified rhematoid nodes in lungs.

The wedge, signs of damage of internals are noted rather seldom. In addition to a pericardis, the changes caused by myocarditis (preferential focal) and an endocarditis can be observed. Cases of formation of heart diseases are known, it is almost exclusive in the form of the isolated insufficiency mitral (left atrioventricular) the valve or valves of an aorta; expressiveness of heart diseases usually small, disturbances of a hemodynamics arise seldom. Damage of lungs at R. and. includes a diffusion fibroziruyushchy alveolitis, nodular defeat of pulmonary fabric (fig. 6), a vasculitis. In some cases it is noted polyserositis (see), to-ry a thicket it is shown pleurisy (see), pericardis (see).

Damage of kidneys (glomerulonephritis) meets much more less than the amyloidosis and medicinal a nephropathy, and is characterized by a moderate proteinuria and a microhematuria.

The diagnosis

Any separately taken symptom is not specific to R. and. Only a combination such a wedge, signs as damage of preferential small joints of brushes and feet, symmetry, the erosive nature and firmness of arthritis, an originality of extraarticular manifestations (hypodermic small knots, a polyneuropathy), and also average and high credits of rhematoid factors in blood serum allow to diagnose R. with big reliability and. For R.'s diagnosis and. at early stages of a disease it is possible to use the diagnostic criteria offered by the American rheumatological association. 11 following symptoms and signs concern to them: 1) morning constraint; 2) pain at the movements or morbidity at least one joint; 3) swelling of a joint; 4) the swelling at least one more joint found with an interval no more than 3 months; 5) symmetry of swelling of joints; 6) hypodermic small knots; 7) typical rentgenol. changes of joints, including circumarticular osteoporosis; 8) detection of rhematoid factors in blood serum; 9) inherent R. and. changes of synovial fluid; 10) characteristic changes of a synovial membrane of the joint capsule; 11) existence of rhematoid small knots characteristic gistol. structures (the symptoms designated by figures 1 — 5, shall be observed during not less than 6 weeks).

In the presence of seven symptoms establish the diagnosis of a classical pseudorheumatism, five — a certain pseudorheumatism, three — probable R. and. For diagnosis of R. and. according to the specified criteria it is also necessary to exclude previously a number of diseases of joints and other diseases, for to-rykh damage of joints is characteristic (see. Joints ).

For R. and. the lab is characteristic a number of disturbances. indicators. At the prevailing most of patients increase in nonspecific indicators of an inflammation is noted: acceleration of ROE, increase in content of fibrinogen, seromucoid, alpha 2 - globulins of blood serum, etc., an also emergence C-reactive protein (see). These changes come to light, as a rule, already at the beginning of a disease, and their expressiveness usually depends on activity of process. In many cases normotsitarny hypochromia anemia is observed (see. Iron deficiency anemia ), degree a cut usually also corresponds to activity of a disease.

For R. and. a row immunol is typical. disturbances, first of all existence in 70 — 80% of cases of rhematoid factors in blood serum and synovial fluid. Frequency of detection of rhematoid factors increases in process of progressing of a disease. In a wedge, practice rhematoid factors define by reaction the Ox of an er — Rouza (add the erythrocytes of a ram loaded with native gamma-globulin of a rabbit to blood serum of the patient), latex test (use of particles of latex with the gamma-globulin of the person adsorbed on them), and, more rare, tests with Dermatolum, to-ruyu carry out as latex test, but instead of latex use particles of Dermatolum. At sick R.' number and., hl. obr. with high activity and a generalized vasculitis, antinuclear reactions, single LE-klet-ki, antinuclear factors and antibodies to native DNA come to light (usually — it is changeable, and in the presence — low credits).

Synovial fluid (see) at R. and. it is characterized by light body, existence of hl in it. obr. neutrophils (to 50% of total number of leukocytes, components usually no more than 10 000 in 1 mkl), nek-ry of them — so-called ragotsita — contain inclusions in the form of the clusters of grapes representing fagotsitirovanny cell-bound immune complexes. Also rhematoid factors are defined, to-rye in synovial fluid earlier, than in blood serum, and also decrease in level of a complement, content of glucose and increase in nek-ry lizosomalny enzymes sometimes come to light.

Help in R.'s diagnosis and. renders morfol. a research of pieces of the synovial membrane of joints received at puncture biopsies (see) or synovectomies (see). To characteristic gistol. to signs of a rhematoid synovitis belong: rhematoid small knots, the expressed proliferation of sinoviotsit with a palisadoobrazny arrangement of cells, diffusion infiltration by lymphocytes, macrophages, plasmocytes, lymphoid follicles, an angiomatosis, a large number of fibroblasts in synovial own plate.

Radiodiagnosis. Enters problems of radiodiagnosis, in addition to recognition of a disease, also assessment of character, weight and prevalence of changes of the bone and joint device, definition of a stage, and at repeated researches assessment of evolution of a disease. The most widespread technique rentgenol. researches the standard X-ray analysis of joints is. More detailed image of structure of a bone can be received, using a screenless X-ray analysis. Tomography (see) at R. and. it is applied extremely seldom and has only auxiliary value. As the most characteristic of R. and. rentgenol. changes are found in adults in brushes and distal departments of feet, and in children, besides, and in cervical department of a backbone, these areas investigate first of all. Dynamics rentgenol. changes at R. and. differs in slow speed that does excessive frequent repetition of a research.

Duration of the X-ray negative period at R. and. considerably varies and most often makes from several weeks to 1 — 2 month. Symmetry of changes of joints and tendency of these changes to progressing are inherent to a pseudorheumatism. The revealed radiological changes unevenly progress, accelerating at exacerbations of a disease and being slowed down, up to full stabilization, during the periods of remissions. At a number of patients correlation between a wedge, disease and progressing rentgenol is not noted. changes, in particular rentgenol. the picture can worsen at absence a wedge, signs of activity of a pseudorheumatism.

At R. and. are found various rentgenol. symptoms, any of to-rykh cannot be considered pathognomonic. Early rentgenol. changes at R. and. are not specific since are inherent to all to arthritises (see), are also characterized by a thickening of soft tissues in the affected joints and circumarticular osteoporosis (see). At an early stage more than at sick R.' half and. also cystic enlightenments in the sites of bones, next to joints, are noted.

Fig. 7. The roentgenogram of distal departments of feet it is normal (and) and at the 3rd (according to Staynbrokker's scheme) of a stage of a pseudorheumatism: unlike norm in fig. osteoporosis and a cystic osteoporosis (1) would be noted circumarticular, joint cracks of metatarsophalangeal joints are narrowed (2), regional erosion of bones preferential in the IV—V area of metatarsophalangeal joints are visible (3).
would be noted by Fig. 8 circumarticular. The roentgenogram of brushes it is normal (and) and at the 3rd (according to Staynbrokker's scheme) of a stage of a pseudorheumatism: unlike norm in fig. osteoporosis (1), narrowing of joint cracks (2), regional erosion of bones in joints (3) would be noted circumarticular,
Fig. 9. Roentgenograms of brushes at the 4th (according to Staynbrokker's scheme) stages of a pseudorheumatism: and — widespread osteoporosis, drying of interarticular cracks, the expressed destructive changes, including ossifluence of proximal interphalangeal joints (are specified by shooters); in fig. along with it the bone anchylosis of all intercarpal joints would be noted (it is specified by an arrow).

Further narrowing of joint cracks, erosion of bones, incomplete dislocations and dislocations (fig. 7, 8) joins. Often against the background of the described changes on regions of joint surfaces osteophytes — display of secondary arthrosis form. Crushing rhematoid damage of joints can lead to their ankiloziro-vaniye; in cases of especially adverse current ossifluence of the bones (fig. 9), next to joints of departments, is possible. As rare rentgenol. symptoms meet periostites (see), aseptic osteonecroses (see. Osteochondropathy ).

On the basis rentgenol. changes of joints allocate R.'s stages and. From numerous schemes Staynbrokker's scheme is most known, according to a cut allocate 4 stages of a pseudorheumatism. Carry cases with circumarticular to the 1st stage osteoporosis (see), but without narrowing of joint cracks and an erosion of bones. If narrowing of joint cracks joins an oyeteoporoz, then it corresponds to the 2nd stage. Existence of erosion of bones indicates the 3rd stage, and a bone anchylosis — the 4th stage. Larsen (A. Larsen, 1974) offered more detailed assessment, having allocated 6 stages, and gave the separate scheme reflecting local features of manifestations of R. for each joint and.

Differential diagnosis presents difficulties usually only at early stages of R. and. at absence rentgenol. symptoms of erosive arthritis and rhematoid factors in blood serum. Most often the differential diagnosis is carried out with nek-ry options of such diseases as Bekhterev's disease, a disease of Reuters, psoriasis arthritis, an osteoarthrosis, gout (see).

Damage of peripheral joints at Bekhterev's disease (see. Bekhtereva disease ) in some cases, however, slight (demanding purposeful search) or short-term.

Damage of joints at psoriasis arthritis (see. Arthritises ), as a rule, differs from R. and. asymmetry, existence of periartikulyarny hypostasis, involvement in process of distal interphalangeal joints of brushes. Detection of typical changes of skin is important. However they can be non-constant, small by the sizes and be localized, e.g., only on a pilar part of the head or nails that at insufficiently attentive survey can lead to a diagnostic mistake.

A secondary synovitis at the deforming osteoarthrosis (see. Arthroses ) with preferential damage of joints of brushes differs from R. and. to-rye are most often involved by what arises only in those joints in process at an osteoarthrosis (distal, is more rare than proximal interphalanx), and practically does not meet in the metacarpophalangeal joints which are usually suffering from R. and., and also lack of rhematoid factors.

Treatment

Exists a large number of various methods of treatment of R. and., the choice to-rykh depends on a form of a disease, degree of its activity, a current, the nature of changes of joints, brought authorized manifestations and other factors. In treatment pharmaceuticals have major importance.

At absence a wedge, signs of extraarticular manifestations, as a rule, it is necessary to begin treatment with selection of non-steroidal anti-inflammatory drugs. From drugs of this group use acetilsalicylic to more often - that, Butadionum, indometacin, an ibuprofen, Voltarenum, Naproxenum — high-speed means, the maximum therapeutic effect to-rykh is shown in 2 — 3 weeks. By consecutive purpose of several drugs select the most effective and well transferred by the patient. In most cases non-steroidal anti-inflammatory drugs use almost continuously in combination with any other means.

Widely apply intra joint introduction of corticosteroids (suspension of a hydrocortisone and drugs of the prolonged action — Kenalogum, depomedrol) that is especially reasonable at defeat of a small number of generally large joints. Purpose of corticosteroids is inside absolutely shown only at patients with a generalized vasculitis, especially at high temperature reaction. Drug of the choice is Prednisolonum, a dose to-rogo select individually (usually 20 — 30 mg, are up to 60 mg more rare in days). In all other cases use of corticosteroids is forced and that is especially important, a temporary measure. Need for it arises, as a rule, at multiple damage of joints with the expressed pain syndrome, to-ry it is not possible to suppress by means of non-steroidal anti-inflammatory drugs p intra joint introduction of corticosteroids. The daily dose of Prednisolonum in such cases shall not exceed 10 mg. Treatment by corticosteroids at R. and. in itself does not give the chance to control adequately the phenomena of arthritis and to slow down progressing of process. During the subsiding of activity of a disease it is necessary to try to lower gradually in all cases a dose and to completely cancel corticosteroid drug, having replaced it with the antirheumatic means possessing slow action.

Preservation expressed a wedge, manifestations and progressing of a disease despite treatment by non-steroidal anti-inflammatory drugs is the indication for use of antirheumatic means of slow action (so-called basic means). Derivatives of quinoline (delagil, plaquenil), salts of gold concern to them (Crysanolum), D-Penicillaminum, levamisole and cytostatic immunodepressants. Due to the slow development of effect (clear signs of improvement appear only in 3 months, and the maximum effect — not earlier than in 6 months of continuous treatment) it is necessary to apply these means it is long. At the same time it is regularly necessary to control the course of treatment since they quite often cause various, sometimes serious side reactions. Choose this or that means of this group, as a rule, randomly (except immunodepressants), only existence of contraindications to use of specific drug matters. Delagil is appointed on 0,25 g a day. At the same time 1 time in 3 — 4 months is necessary survey of the patient with the oculist for the prevention of complications from an organ of sight. Crysanolum is entered intramusculary 17 — 51 mg on an injection (the ampoule of 5% of solution of Crysanolum contains 34 mg of crystal gold). D-Penicillaminum is appointed on 300 mg a day within the first 3 months, then in the absence of effect each 4 weeks increase a daily dose by 150 mg. The maximum daily dose of drug usually shall not exceed 750 — 900 mg. At use of Crysanolum and D-Penicillaminum it is necessary to conduct weekly researches of urine and blood for the purpose of diagnosis and the prevention of development of a medicinal nephropathy and a cytopenia. Levamisole is appointed on 150 mg a day within one or three consecutive days every week or daily with an obligatory weekly research of number of leukocytes of blood.

At considerable improvement of a condition of the patient in 6 — 8 months after an initiation of treatment it is reasonable to continue treatment during that time term (sometimes years) while the reached effect remains. In the absence of a positive take within 6 — 8 months appoint other drug of this group.

Cytostatic immunodepressants also belong to slowly operating means, but Apply them usually in the last turn. However in some cases at a generalized vasculitis, a polyneuropathy, the expressed signs of damage of internals (nephrite, an alveolitis) immunodepressants are drugs of the choice. Use usually Chlorbutinum (a daily dose of 8 — 12 mg), Azathioprinum and, more rare, cyclophosphamide (100 — 150 mg a day). At the same time once a week carry out bulk analysis of blood.

Is of great importance to lay down. physical culture, classes a cut shall be given daily and are limited only at high activity of a disease and considerably the expressed pain syndrome. During the subsiding of activity of R. and. to most of patients it is shown massage (see).

Physical methods of treatment apply in complex therapy of R. of ampere-second the purpose of local impact on inflammatory process in joints, activation of regulatory systems and processes of recovery. The physical therapy is shown in any stage of R. and. at the minimum or average activity of a disease, lack of bright extraarticular manifestations. The inductothermy and decimeter waves have antiinflammatory effect, to-rye apply on area of joints at the insignificant exudative phenomena. At the expressed exudative reaction in joints antiinflammatory effect is had Uv-radiation, electrophoresis (see) and fonoforez (see. Ultrasonic therapy ) anti-inflammatory drugs. Inductothermy (see) the affected joints carry out by means of the inductor (a cable or a disk) in a slaboteplovy or thermal dose within 10 — 15 min. (no more than 30 — 35 min. on one procedure). At treatment by decimeter waves (see. Microwave therapy) a radiator have at distance of 3 cm from the surface of skin in a joint and use a thermal dose, lasting 7 — 14 min. The course of treatment consists usually of 12 — 18 procedures.

For the purpose of stimulation of synthesis of glucocorticosteroids adrenal glands apply impact of an inductothermy and decimeter waves on area of a projection of adrenal glands (level of the lower chest and lumbar departments of a backbone, Th10 — L4) in the same doses, as on area of joints.

UF-radiation is recommended to carry out in erythema doses (5 — 6 procedures). At the expressed exudative phenomena apply fonoforez suspensions of a hydrocortisone or the electrophoresis of analginum (2 — 5% solution), at to-rykh antiinflammatory effect of pharmaceuticals is combined with action of ultrasound or direct current. Appoint applications with a dimethyl sulfoxide (see), to-ry has antiinflammatory effect, promotes deeper penetration through skin of the pharmaceuticals applied in mix with it, napr, heparin, analginum. At dominance of proliferative changes in joints by the patient with the minimum activity of process ultrasonic therapy is shown (intensity of 0,2 — 0,6 W/cm 2 , 4 — 6 min. on a joint, on a course 8 — 12 procedures). Ultrasonic therapy at high activity and system manifestations of R. is contraindicated and., and also the accompanying coronary heart disease and a thyrotoxicosis. Resorptional action of a dimethyl sulfoxide on fibrous fabric is used for treatment of flexion contractures. This method is contraindicated at an allergy to drug.

At dominance of fibrous changes of joints and muscular contractures carry out applications of dirt (see Mud cure), ozokerite (see the Ozoceritotherapy) and paraffin (see. Paraffin therapy ).

The patient with the minimum activity of R. and. the balneoterapiya is shown (see): radonic bathtubs (40 — 80 nkyuri/l), sulphidic (concentration of hydrogen sulfide of 50 — 100 mg/l), chloride sodium (20 g/l), iodine-bromine, and also naftalanovy bathtubs with water temperature 36 — 38 ° lasting from 8 to 15 min. (on a course 8 — 12 procedures). Balneoterapiya and a thermotherapy are contraindicated at high activity of process, existence of the expressed exudative phenomena in joints, extraarticular manifestations, and also at the general contraindications to separate types of a balneoterapiya and a thermotherapy.

Surgical correction of changes of joints at R. and. make usually only in late stages of a disease, at development of considerable functional disturbances — flexion contractures, incomplete dislocations, ankiloz of joints. The choice of a method of operational treatment depends on the nature of damage of separate joints: synovectomy (see), prosthetics of joints (see. Endoprosthesis replacement ), artificial ankylosis (see), etc. At mono - oligoartrita the so-called early synovectomy which is carried out or an operational way, or by means of intra joint introduction of radioactive nuclides is effective (gold, yttrium, etc.).

The forecast for life in most cases favorable. According to Reygen and Farrington (S. of Ragan, E. Farrington, 1964), the worst forecast concerning a functional condition of joints is noted when R. and. begins aged up to 30 years, at remaining R.'s activity and. within more than one year from an onset of the illness, in the presence of hypodermic rhematoid small knots and a high caption of rhematoid factors in blood serum. The forecast worsens also at emergence in the patient a wedge, signs of a vasculitis, development of a syndrome of Shegren, an amyloidosis.

A juvenile pseudorheumatism

the Juvenile pseudorheumatism (a synonym juvenile chronic arthritis) — the special form of a pseudorheumatism which is developing at children aged up to 16 years and characterized by the acute beginning, fever, extraarticular manifestations, defeat of a small number of joints (mono - an oligoarthritis), preferential large (knee, talocrural), and frequent involvement of joints of cervical department of a backbone, and also frequent development uveitis (see), rare detection of rhematoid factors in blood serum, more favorable current and the forecast.

Juvenile R.'s prevalence and., according to a number of researchers, makes 0,01 — 0,001% among the children's population. At children of chest age cases are rare; more often (by 1,5 — 2 times) girls are ill.

Etiology and pathogeny are studied insufficiently and are considered usually from the same positions, as at R. and. at adults. However at juvenile R. and. certain genetic features, in particular distribution of histocompatibility antigens attract attention (see. Immunity transplant ). So, according to Fink and Stastni (Page W. Fink, P. Stastny, 1979), at juvenile R. and. less than in population, and less than at R. and. at adults, HLA Dw 4 is defined and HLA TMO is much more often, especially at mono - and oligoartikulyarny options. At monoarthritis and oligoartikulyarny option HLA Dw 7 and Dw 8 also often are defined. In comparison with R. and. at adults at juvenile R. and. extremely seldom HLA DRw 4 comes to light.

These data, as well as features of immune disturbances, a current and the forecast of a disease, induce a number of scientists to discuss a question of his nosological independence. On the other hand, there is an opinion that juvenile R. and. represents combined group of chronic inflammatory diseases of joints of various etiology. It is based hl. obr. on the known difficulties of differential diagnosis at children of the diseases proceeding with damage of peripheral joints, napr, Bekhterev's disease, psoriasis, chronic enteropathies.

In this regard at the International working meeting pediatricians-roar-matologov (1977) it was offered to give preference to the term «juvenile chronic arthritis».

The nature of fabric damages at juvenile R. and. in general does not differ from the described changes at R. and. at adults. However at children note weaker hyperplastic cellular reaction and inflammatory infiltration in a synovial membrane, find erosion in a joint cartilage less often.

The main signs of damage of joints at juvenile R. and. same, as well as at R. and. at adults: pain, swelling, morning constraint. Expressiveness of joint pains at children usually is less, than at adults, sometimes, despite a swelling of a joint and its morbidity at a palpation, complaints to pains in general are absent. Children of younger age seldom complain of morning constraint, to-ruyu it is possible to reveal only at attentive overseeing them behavior. Such general symptoms as weakness, increased fatigue and irritability, loss of appetite, weight loss, subfeb-rilny temperature are quite often noted.

At juvenile R. and. arthritis of small joints of brushes and feet is less often noted. Approximately at 1/3 patients involvement of intervertebral joints of cervical department of a backbone (especially C2 — C3) with pains and restriction of movements is observed. Arthritis is often noted temporal and mandibular joints (see). Approximately at 25% of patients usually in several years from the beginning of a disease at rentgenol. a research comes to light sacroileitis (see). In general destruction of a joint cartilage and adjacent departments of bones and deformation of joints develop at juvenile R. and. much more slowly, than at R. and. at adults.

Fig. 10. Sick with a juvenile pseudorheumatism with an underdeveloped mandible (microgenia).

Characteristic features of juvenile R. and. delay of physical development, lag in growth, and also growth disorder of separate segments of a skeleton in connection with damage of nearby joints, napr, an underdevelopment of a mandible — a microgenia (fig. 10), shortening or lengthening of phalanxes of fingers or other bones are.

It is accepted to allocate three a wedge, option of the beginning of juvenile R. and. (on set of the symptoms developing within 4 — 6 months of a disease): polyarticulary, mono - oligoartikulyarny and system.

Polyarthritis (see) from the very beginning diseases it is noted approximately at 50% of patients. Polyarticulary option to a large extent, than others, on-lominayet R. and. at adults, but the number of the affected joints usually are less; at many patients it is noted spopdiloartrit (see) cervical department of a backbone. More often than at other options, rhematoid factors come to light (see. Rhematoid factor). Brought authorized manifestations meet infrequently, their expressiveness much less, than at system option. Disease is wavy, more or less long remissions are frequent, but at a number of patients gradual progressing of a disease is noted.

Mono - oligoartikulyarny option of the beginning of juvenile R. and. it is noted approximately at 1/3 patients. Preferential knee, ankle, wrist joints are surprised. At many patients long time can be observed monoarthritis any of these joints (more often than knee). Also poorly expressed exudative changes of joints, rarity of defeat of cervical department of a backbone, system manifestations (except for a uveitis) and emergence of a rhematoid factor in blood serum, the minimum deviations from outside a lab are characteristic moderately. indicators testimonial of an inflammation. Approximately at 20% of patients with this option of the beginning of juvenile R. and., as a rule at girls, in blood serum the antinuclear factor comes to light. To their thicket the uveitis develops. Eventually at 50 — 60% of patients moderately expressed oligoarthritis remains, involvement of new joints is not noted. At 25% of patients of this group the long remissions alternating with rather short aggravations reminding an onset of the illness are observed; the others have a gradation in polyarticulary option.

System option of the beginning of juvenile R. and. it is characterized by high fever, existence of rhematoid rash, a gepatosplenomegaliya, generalized lymphadenopathy, a serositis, the expressed leukocytosis in blood and carries the name of a syndrome of Steel. At nek-ry children arthritis develops only in several weeks or even months after the beginning of fever and other extraarticular displays of a disease. Rhematoid factors usually do not come to light, antinuclear antibodies meet seldom, LE cells, as a rule, are not found. Approximately at a half of patients with system option of the beginning of juvenile R. and. further long remissions with the periodic aggravations which are characterized by the same manifestations, as in the beginning are noted. At others for a long time the disease remains active, as a result of treatment it is possible to reduce expressiveness of extraarticular manifestations, however the progressing damage of joints becomes the main problem. System option of the beginning of juvenile R. and. can proceed as Vissler's subsepsis — Fankoni (see. Subsepsis allergic ).

The most serious brought authorized display of a disease — the chronic uveitis (which is almost not found at R. and. at adults), in some cases bringing to to a blindness (see). The uveitis develops at girls with oligoartikulyarny option of a disease in the presence in blood serum of antinuclear antibodies more often. Damage of eyes quite often proceeds without bright a wedge, symptoms, sometimes asymptomatically and has no parallelism with expressiveness of joint displays of a disease.

At 10 — 20% of patients high temperature (38 — 39 °) from oznoba is observed. Fever has intermittent character, rises in temperature are noted 1 — 2 time a day, is more often in the second half of day; it does not decrease at treatment by antibiotics and gives in only to high doses acetilsalicylic to - you and especially to glucocorticosteroids. As a rule, high temperature is accompanied rhematoid (pinkish color) by the rash having korepodobny makulezny character, which is localized preferential on a trunk and proximal departments of extremities (tsvetn. fig. 6). This rash is specific to juvenile R. and. and its detection has great diagnostic value. Rhematoid rash quickly passes, «blossoms» in a rising period and at a potiraniye of suspicious sites of skin (see. Kebnera phenomenon ).

Typical extraarticular manifestations of juvenile R. and. an exudative pericardis (other manifestations of a serositis — pleurisy, peritonitis are more rare), the generalized lymphadenopathy, increase in a liver, sometimes spleens are. The pericardis is poorly expressed to a thicket. Extraarticular manifestations (except a uveitis) meet, as a rule, at system option and are much more rare at polyarticulary and oli-goartikulyarny options of a disease.

From other system manifestations at juvenile R. and. myocarditis, an endocarditis (with slow development of insufficiency of the mitral valve and valves of an aorta), the diffusion intersticial pulmonary fibrosis, a glomerulitis (which is shown only a non-constant microhematuria) are described. Hypodermic small knots, polyneuropathy, digital vasculitis so characteristic of R. and. at adults, meet only in 5 — 10% of cases, usually in the presence of rhematoid factors in blood serum.

At a number of patients with a juvenile pseudorheumatism usually in many years from an onset of the illness the amyloidosis, hl can develop. obr. kidneys.

Fig. 11. The roentgenogram of cervical department of a backbone at a juvenile pseudorheumatism (a side projection): the arrow specified a bone anchylosis of vertebral joints in the area III and IV of cervical vertebrae.

At juvenile R. and. the same are observed rentgenol. changes, as at R. and. More frequent is characteristic, than at R. and., development of arthritis of small joints of cervical department of a backbone, sometimes from the outcome in an anchylosis (fig. 11), and disturbances of normal process of a skeleton: acceleration of growth of tubular bones of the affected extremity or, on the contrary, an underdevelopment of separate bones (e.g., a mandible).

Changes lab. indicators are not specific. At many patients normotsitarny hypochromia anemia, acceleration of ROE, increase in levels of fibrinogen, osg-globulins in blood serum, emergence of S-reactive protein is noted. These changes usually correlate with activity of a disease. Also neutrophylic leukocytosis which is especially expressed is usual (to 15 000 — 50 000 in 1 mkl) at system option of a disease. Rhematoid factors in blood serum come to light no more than at 20% of patients, as a rule through a long term from an onset of the illness. At 40% of patients the antinuclear factor is defined (usually changeably and in small credits). Existence of antibodies to native DNA and LE cells to juvenile R. and. unusually. The nature of changes of synovial fluid in general same, as well as at R. and. at adults, however in it rhematoid factors and ragotsita meet less often.

As at juvenile R. and. are absent specific clinical (except for seldom found rhematoid rash) and a lab. signs, the diagnosis of a disease with confidence it can be established only in the presence of persistent arthritis and an exception of other diseases having similar manifestations. The group of rheumatologists of the socialist countries in 1979 offered the following criteria, to-rye it is necessary to consider at early diagnosis of a juvenile pseudorheumatism: 1) arthritis lasting more than 3 weeks (obligatory sign); 2) damage of three joints during the first 3 weeks of a disease; 3) symmetric damage of small joints; 4) defeat of cervical department of a backbone; 5) signs of an exudate in a joint cavity; 6) morning constraint; 7) tendosinovit or a bursitis; 8) uveitis; 9) rhematoid small knots; 10) epiphyseal osteoporosis; 11) narrowing of a joint crack; 12) consolidation of periartikulyarny fabrics (signs 10, 11 and 12 establish at rentgenol. research); 13) acceleration of ROE more than 35 mm an hour; 14) detection of rhematoid factors in blood serum; 15) characteristic gistol. the data obtained at a biopsy of a synovial membrane.

With three criteria the diagnosis is considered probable, four — certain, seven — classical; in all cases an indispensable condition is existence of the first criterion.

Differential diagnosis carry out first of all with rheumatism (see), system lupus erythematosus (see) and Bekhterev's disease.

At rheumatism of a joint pain usually very strong, the migrating character, short (it is noted expressed to lay down. effect of use acetilsalicylic to - you), fever usually the constant without oznob, rash has character of a ring-shaped erythema, substantial and permanent increase of antistreptococcal antibodies in blood serum is noted. At juvenile R. and. unlike rheumatism emergence of loud noise in heart is not observed.

For a system lupus erythematosus unlike juvenile R. and. the polisindromnost is typical (an erythema on face skin, a diffusion glomerulonephritis, signs of defeat of c. N of page, etc.), existence of LE cells in blood, a significant amount of antibodies to native DNA, decrease in level of a complement.

Bekhterev's disease develops preferential at boys of school age, the main differential sign — a prematurity of a bilateral sacroileitis, existence of HLA B27.

During the carrying out differential diagnosis take into account also a possibility of development of arthritis at nek-ry acute viral infections, napr, a rubella, measles, an infectious mononucleosis, hepatitis B, etc., and also sepsis, tuberculosis, an acute leukosis and lymphoma, hemophilia, an injury, hereditary immunodeficient diseases (hypo - and an agammaglobulinemia, deficit of immunoglobulin A, C2 of a component of a complement), etc.

Treatment carry out by the same means and methods, as R. and. at adults. Only levamisole and D-Penicillaminum at juvenile R. and. did not find broad application in connection with frequent development of serious complications. At many patients in treatment it is possible to be limited to non-steroidal anti-inflammatory drugs and intra joint introduction of a hydrocortisone. Derivatives of quinoline and salt of gold are appointed at oligoartiku-lyarny and polyarticulary options of an onset of the illness in case of insufficient effect of the previous treatment and lack of tendency to spontaneous remissions. Corticosteroids inside and immunodepressants apply seldom, usually only at system option and, as a rule, during a limited span (several months).

Early detection and persistent treatment of a uveitis is of great importance. At oligoartikulyar-number option of the beginning of R. and. a year the oculist shall examine children, especially girls, 2 — 3 times. In some cases, in addition to topical treatment, appointment in corticosteroids is shown.

Forecast in general favorable. At most of patients long remissions are noted, expressiveness of arthritis for many years can remain small, the number of the affected joints, as a rule, is limited, considerable functional disturbances develop seldom. But approximately 1/3 patients as a result of the chronic progressing arthritis gradually have essential restrictions of movements in joints, contractures and ankiloza develop. Especially often it is noted at patients with sistekhmny and polyarticulary options of the beginning of a disease. Sharply development of a uveitis (predictive value has detection of an antinuclear factor in blood serum) and secondary worsens the forecast amyloidosis (see).



Bibliography: Dolgopolova A. V., etc. Development of criteria of early diagnosis of a juvenile pseudorheumatism, Vopr. revm., No. 4, page 3, 1979; Dormidontov E. N., N. I. Kites and F r and z e B. N N. Pseudorheumatism, M., 1981; Spear-in and T. N. Pathology of a pseudorheumatism, M., 1980, bibliogr.; Nasonova V. A. The pseudorheumatism in modern rheumatology, Rubbed. arkh., t. 50, No. 9, page 3, 1978; Nasonova V. A. and d river. Pharmacology in rheumatology, M., 1976; Olefirenko V. T. Vodotepl about treatment, page 121, 234, M., 1978; Pavlov V. P. Complex surgical treatment of infectious nonspecific polyarthritis, M., 1977; Rhematoid polyarthritis, under the editorship of N. S. Kosinskaya, page 52, JI., 1967; Strukov A. I. New aspects of the doctrine about an inflammation (an immune inflammation), Arkh. patol., t. 43, No. 1, page 3, 1981, bibliogr.; Strukov A. I. and B e of l and r I am a N And. G. Pathological anatomy and pathogeny of collagenic diseases, M., 1963; To the Container ev E. M. and d river. Collagenoses, M., 1965; Yakovleva A. A. Diseases of joints at children's age, M., 1977; Bankhurst A. D., Husb at G. a. Williams R. Page of Predominance of T cells in the lymphocytic infiltrates of synovial tissues in rheumatoid arthritis, Arthr. and Rheum., v. 19, p. 559, 1976; D u-t h i e J. J. a. o. Course and prognosis in rheumatoid arthritis, Ann. rheum. Dis., v. 23, p. 193, 1964; Passben-d e r H. G. Pathologie rheumatischer Erkrankungen, B. — N. Y., 1975, bibliogr.; N a t v i g J. B. a. o. Lymphocyte subpopulations and immune reactions in rheumatoid inflammatory tissue, Scand. J. Rheumatol., v. 5, suppl. 12, p. 77, 1976; Rheumatic diseases, ed. by W. A. Katz, p. 385, 446, Philadelphia, 1977; S o-r e n A. Histodiagnosis and clinical correlation of rheumatoid and other synovitis, Stuttgart a. o., 1978; Stastny P. a. Fink C. W. Different HLA — D associations in adult and juvenile rheumatoid arthritis, J. clin. Invest., v. 63, p. 124, 1979; Steinbrocker O. O., T r an e-ger C. H. a. Batterman R. C. Therapeutic criteria in rheumatoid arthritis, J. Amer. med. Ass., v. 140, p. 659, 1949; Textbook of rheumatology, ed. by W. N. Kelly a. o., Philadelphia a. o., 1981; Wager O. Immunopathogenic mechanisms in rheumatoid diseases with special reference to IgM rheumatoid factor, Scand. J. Rheumatol., v. 5, suppl. 12, p. 67, 1976, bibliogr.


V. A. Nasonova, H. V. Bunchuk; V. D. Grigorieva (fizioter.), T. N. Kopyeva (stalemate. An.), H. M. Mylov (rents.).

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