LUPUS ERYTHEMATOSUS

From Big Medical Encyclopedia

LUPUS ERYTHEMATOSUS (lupus erythematodes, lupus erythematosus; synonym: erythema centrifugum, eritematoz) — the group concept including a number of nosological units, hl. obr. system lupus erythematosus and diskoidny lupus erythematosus, and also syndrome of a medicinal lupus (drug-induced lupus). System and diskoidny To. have a number of common features century. So, both system, and diskoidny To. strikes preferential women century; an erythematic enanthesis of the person, extremities, trunks and mucous membranes (enantema), hypersensitivity to solar radiation are characteristic of both forms (photosensitization); transition diskoidny is possible To. century in system (at 3 — 5% of patients); in separate families patients diskoidny, system can meet To. century and other collagenic diseases. At the same time, distinctions in the nature of erythematic rashes and especially system manifestations at system and diskoidny To. century, features of a pathogeny, in particular deep disturbances of an immunogenesis at system To. century, allow most of authors to consider them as separate nozol, forms. It found reflection in «Statistical classification of diseases and causes of death» (1969): diskoidny To. belongs to the class XII «Disease of skin and hypodermic cellulose» century, and system To. century — to a class XIII «Disease of a musculoskeletal system and connecting fabric».

A system lupus erythematosus

System To. century (lupus erythematosus systemicus; synonym: acute lupus erythematosus, erythematic hroniosepsis, Liebman's disease — Saks) — a chronic general inflammatory disease of connecting fabric and vessels with the expressed autoimmune pathogeny and, apparently, a virus etiology; treats diffusion diseases of connecting fabric — to collagenoses (see. Collagenic diseases ). System To. century — a disease of women of childbearing age (20 — 30 years), girls of teenage age quite often get sick. A ratio of the women and men having this disease on the majority the statistician — 8: 1 — 10: 1.

History

System To. it is described in 1872 by the Vienna dermatologist of M. Kaposha as diskoidny century To. century, characterized by fever, a pleuropneumonia, bystry development of a coma or sopor and death. In 1923 Liebman and Saks (E. Libman and V. of Sacks) described atypical verrukozny an endocarditis (Liebman's endocarditis — Saks), a polyserositis, pneumonia and erythematic rashes in the field of a ridge of the nose and zygomas — a so-called butterfly. The Sovvremenny doctrine about system To. it is connected with names of Klemperer, Pollack and Rem century (P. Klemperer, A. D. Pollack and G. Baehr) which in 1941 drew attention to a diffusion collagenic disease (diffuse collagen disease), having described systemic lesion of connecting fabric at this disease and a scleroderma. With opening of LE cells (Lupus erythematosus cells) by Hargreyvs, Richmond and Moreton (M. M. of Hargraves, H. Richmond, R. Morton) in 1948, and in 1949 Heyzerik (Y. R. Na-serick) a lupoid factor paid attention to autoimmune disorders.

In domestic literature the first the wedge, the description of «an acute lupus erythematosus» belongs to G. I. Meshchersky (1911), and a patomorfologiya — to I. V. Davydovsky (1929), etc. Systematic studying system To. century in our country E is begun. M. Tareev, O. M. Vinogradova, etc. In 1965 E. M. Tareev with soavt, in monographs «Collagenoses», having analyzed 150 observations, described system To. century in all its variety, raised a question of a curability of a disease and planned ways of further studying. Unconditional progress in development of the doctrine about system To. it is caused by highly effective treatment by corticosteroids and immunodepressants century.

Statistics

Population researches of Ziegel (M, Siegel) et al. (1962 — 1965) showed that incidence near Manhattan (New York) increased with 25 on 1 million people in 1955 to 83 on 1 million in 1964 Dubois (E. L. Dubois, 1974) assumes that in the USA system To. century annually 5200 people get sick, therefore, each 5 years al least 25 000 patients collect. Leonkhardt (T. Leonhardt) in 1955 showed that a prevalention system To. century in. To a bulltrout (Sweden) from 1955 to 1960 was 29 on 1 million. Mortality in the USA, according to Kobba (Cobb, 1970) — 5,8 on 1 million population, is higher among women at the age of 25 — 44. The lethality, on materials Ying-that rheumatism of the USSR Academy of Medical Sciences, decreased from 90% in 1959 — 1960 to 10% by 1975

the Aetiology

the Aetiology is not found out, however the hypothesis of a role hron, a persistent viral infection gained development in connection with detection at a submicroscopy in the struck bodies (skin, kidneys, a synovial membrane) of the tubuloretikulyarny structures located in cytoplasm of endothelial cells and also in lymphocytes and thrombocytes of peripheral blood which reminded nucleoproteid of paramyxoviruses. At system To. century the circulating antibodies to viruses of measles, a rubella, a parainfluenza and other RNA viruses from group of paramyxoviruses were also found in high credits. At patients and their relatives the limfotsitotoksichesky antibodies which are markers of a persistent viral infection, and, besides, in the same groups and at the medical staff working with patients — antibodies to double-helix (virus) RNA are revealed. Due to the virus etiology system To. century such phenomena as hybridization of a genome of a clumsy virus are discussed with DNA of cells of the struck bodies (a spleen, kidneys), detection of antigens of oncornaviruses of type C in fractions of a spleen, a placenta and a kidney. A hypothesis of the importance hron, a viral infection at system To. is based century also on studying of a disease of the New Zealand mice at whom the role of an oncornavirus of type C is proved.

The intolerance of drugs, vaccines, photosensitization, formation of a menstrual cycle, pregnancy, childbirth, abortions, etc. are considered as the factors provoking a disease or its aggravation; they matter for prevention and timely diagnosis as communication began or exacerbations of a disease with these factors is more characteristic for system To. century, than for other close diseases.

Pathological anatomy

Fig. 1. The diffraction pattern of a kidney at a system lupus erythematosus with tubular educations in an endothelium of glomerular capillaries: 1 — tubular educations; 2 — a mitochondrion; 3 — lysosomes, 4 — a gleam of a capillary; X 70 000.
Fig. 2. A smear print from inflammatory exudate (pneumonic focus) at a system lupus erythematosus: LE cells are expressed (are specified by shooters).

System To. century, being the representative of group of collagenic diseases, it is characterized by generalized distribution patol, the process covering all bodies and systems that causes kliniko-anatomic polymorphism of a disease. Generalization is caused by circulation in blood of the cell-bound immune complexes damaging vessels of a microcirculator bed, the system progressing disorganization of connecting fabric is a consequence of what. Immunopatol. reactions are confirmed by strengthening of function of bodies of an immunogenesis, precipitation in walls of vessels and in the struck fabrics of cell-bound immune complexes with emergence immunocompetent cells (see). Damage of vessels of microcirculation is shown by widespread vasculites of destructive or proliferative character (see. Vasculitis ). In an endothelium of capillaries electronic microscopically reveal peculiar tubular educations (fig. 1) similar to a ribonucleoprotein of a paramyxovirus and, perhaps, players etiol, a role.

Specifics of fabric reactions at system To. cause symptoms of pathology of cellular kernels century: basophilia of fibrinoid, karyorrhexis, gematoksilinovy little bodies, LE cells, central chromatolysis. The basophilia of fibrinoid is caused by impurity of acid products of nuclear disintegration. The Gematoksilinovy little bodies described in 1932 by L. Gross represent the bulked-up kernels of the dead of cells with lizirovanny chromatin. LE cells, or cells of a lupus erythematosus, are mature neutrophils which cytoplasm is almost entirely filled with a fagotsitirovanny kernel of the dead of a leukocyte. Own kernel at the same time is pushed aside to the periphery. They can be met in sine limf, nodes, in smears prints from inflammatory exudate, napr, from pneumonic focuses (fig. 2). The central chromatolysis is shown by washing away of chromatin from the center of cellular kernels with an enlightenment of the last.

Fig. 6. Microdrug of a kidney at a lupoid glomerulonephritis with characteristic signs of a system lupus erythematosus: 1 — focal fibrinoid: 2 - "wire loops"; 3 — hyaline blood clots; 4 — a karyorrhexis.

The most characteristic changes at system To. note in kidneys, heart, a spleen century. Damage of kidneys is characterized by development of the lupoid glomerulonephritis which is microscopically shown in two forms: 1) with characteristic signs system To. century; 2) without characteristic signs system To. century (V. V. Serov and soavt., 1974). Fibrinoid of capillaries of balls, a phenomenon of «wire loops», hyaline blood clots, a karyorrhexis belong to characteristic signs (tsvetn. fig. 6). «Wire loops» call the basal membranes of glomerular capillaries thickened, impregnated with plasma proteins and bared owing to exfoliating of an endothelium which are considered as a prestage of fibrinoid changes. Described them in 1935. G. Baehr and soavt. Hyaline blood clots are located in a gleam of glomerular capillaries and on tinktorialny properties are regarded as intravascular fibrinoid. For the second form development of the hymenoid, hymenoid and proliferative or fibroplastic changes inherent in a banal glomerulonephritis is peculiar. Both forms quite often meet in a combination.

Fig. 3. An immunofluorescence of immunoglobulins (light sites) in renal balls at a lupoid glomerulonephritis; Koons's method.
Fig. 4. The diffraction pattern of a kidney at a lupoid glomerulonephritis. Osmiofilny deposits on a subendothelial surface of a basal membrane of a renal ball: 1 — deposits; 2 — a basal membrane; 3 — an endothelium; 4 — a gleam of a capillary; 5 — a kernel; 6 — shoots of podocytes; x 18 000.

Damage of renal balls by cell-bound immune complexes is the cornerstone of development of a lupoid glomerulonephritis. At immunofluorescent microscopy in balls find a luminescence of immunoglobulins (fig. 3), a complement, fibrin. Electronic microscopically reveal equivalents of cell-bound immune complexes in the form of deposits (fig. 4). At localization of the last on a subepithelial surface of a basal membrane damage of shoots of podocytes, formation of aculeiform outgrowths of a membrane is noted that designate as hymenoid transformation. In clinic at the same time often note a nephrotic syndrome. Proliferative reaction, according to V. V. Serov and soavt. (1974), it is connected with reproduction of mezangialny cells. In the outcome of lupoid nephrite secondary wrinkling of kidneys develops.

Fig. 5. Macrodrug of heart of the person who died from a system lupus erythematosus (in a section), with a polypostural endocarditis of Liebman — Saks (shooters specified warty imposings).

Damage of heart is characterized by development of an endocarditis of Liebman — Saks (fig. 5). The endocarditis affects shutters and chords of valves, a pristenochny endocardium, usually does not lead to heart disease, however development of insufficiency of the mitral valve is possible. In a myocardium find fatty dystrophy of muscle cells («tiger» heart), diffusion proliferative interstitial myocarditis — a lupoid carditis is more rare. Most often the pericardium is surprised.

Fig. 6. Microdrug of a spleen at a system lupus erythematosus: 1 — a gleam of an artery; 2 — a feather an arterial «bulbous» sclerosis.

The spleen is increased, microscopically in it find a characteristic sign — a «bulbous» sclerosis — layered ring-shaped growth of collagenic fibers in the form of the coupling around the sclerosed arteries and arterioles (fig. 6). Follicles are atrophied, in a red pulp plasmatization and Macrophagic reaction are expressed. Plasmatization is noted also in increased limf, nodes, marrow, a thymus.

Development of the lupoid pneumonitis proceeding as intersticial pneumonia with vasculites and cellular infiltration of intersticial fabric is possible. Damage of lungs can be connected with accession of consecutive infection.

Lupoid process can affect a liver. At the same time in portal paths observe limfoplazmotsitarny infiltration, dystrophy of hepatocytes.

Defeat of a nervous system is connected with vasculites.

Visceral defeats are often combined with defeat of a musculoskeletal system and skin. At high activity of a disease in skeletal muscles the picture of an acute focal miositis is defined. In joints the picture of an acute synovitis with dominance of exudative reactions and usually without the subsequent deforming processes can develop.

Fig. 7. Microdrug of skin with a proliferative vasculitis (it is specified by shooters) at a system lupus erythematosus.
Fig. 7. An immunofluorescence of immunoglobulins on a basal membrane of epidermis of skin at a system lupus erythematosus; Koons's method.

At microscopic examination of skin of the struck and externally not affected areas find vasculites in 70 — 80% of patients, is more often proliferative (tsvetn. fig. 7). The immunofluorescent research reveals a luminescence of immunoglobulins on a basal membrane in the field of a dermo-epidermal joint (fig. 7).

Complications and the displays of a disease conducting by the death of patients (the renal failure, focal and drain pneumonia, sepsis, anemia, vasculites leading to heart attacks of a brain, heart) have bright morfol, signs. On morfol. a picture corticosteroid therapy leaves a mark, a cut are a consequence oppression of reaction of bodies of an immunogenesis, an atrophy of adrenal glands, osteoporosis, areactive ulcers went. - kish. a path, signs of a syndrome of Itsenko — Cushing, sometimes the outbreak of tuberculosis, sepsis. Active treatment caused the medicinal pathomorphism of a disease which is characterized by dominance hron, forms of a disease over acute, increase of specific weight of proliferative processes, sclerous changes, decrease in frequency of a karyorrhexis, gematoksilinovy little bodies, Liebman's endocarditis — Saks.

Morfol, diagnosis system To. century positive takes of an immunofluorescence, vasculites, disorganization of connecting fabric, Liebman's endocarditis — Saks are based on the accounting of pathology of kernels, a lupoid glomerulonephritis, «bulbous» sclerosis in a spleen. For an intravital morfol, diagnosis investigate material of a biopsy of kidneys, skin, skeletal muscles with obligatory use of immunofluorescent methods.

A pathogeny

At system To. century the role of disturbances of a humoral link of immunity with development of organonespetsifichesky autoimmune reactions is obvious that it is shown by hyperfunction of B-lymphocytes and a wide range of circulating autoantibodies (see) — to integral kernels of cells and separate ingredients of a kernel (DNA, nucleoproteid), and also lysosomes, mitochondrions, cardiolipids (false positive Wassermann reaction), coagulant factors of blood, leukocytes, thrombocytes, erythrocytes, the aggregated gamma-globulin (see. Rhematoid factor ), etc. These antibodies, being antibodies — witnesses of the happened damage, are capable to create the circulating cell-bound immune complexes which are postponed on basal membranes of kidneys, skin, etc., cause their damage with development of inflammatory reaction. Such is the immune and complex mechanism of development of lyupus-nephrite, vasculites, etc. Existence of the DNA complex — an antibody to this DNA and a complement is proved by allocation from tissue of a kidney of antibodies to DNA, and cell-bound immune complexes are found by method immunofluorescence (see). High activity system To. it is characterized by a gipokomplementemiya century — decrease in maintenance of an integral complement (CH50) and its components, especially C3 which is taking part in reaction antigen — an antibody, C4, Sd1, C9, etc. (see. Complement ). It is saved up many facts specifying that the imbalance of humoral and cellular links of immunity takes place; the last is shown by various reactions of hypersensitivity of the slowed-down type, decrease in maintenance of T lymphocytes. Existence in separate families system and diskoidny To. century, various autoimmune diseases, a photosensitization and intolerance of medicines, detection of broad range of the circulating autoantibodies at members of these families allow to think of a role of genetic predisposition in a course of a disease, however specific mechanisms of this predisposition are not known yet.

Pilot models system To. century — the disease of the New Zealand mice (NZB, NZW and their hybrids of NZB/NZW F1) and dogs of special genetic lines (canine lupus) — is confirmed by the provisions stated above as these models, certainly, are characterized by genetic predisposition, an imbalance in humoral and cellular links of immunity and vertical transfer of an oncornavirus With at the New Zealand mice.

A clinical picture

Complaints of patients are various, but most often they complain of joint pains, fever, disturbance of appetite, a dream. As a rule, system To. begins subacutely with the recurrent polyarthritis reminding rheumatic, temperature increase, various skin rashes, an indisposition, weakness, a lose of weight century. The acute beginning with high temperature, sharp pains and a swelling of joints, a symptom of «butterfly», a polyserositis, nephrite, etc. is less often observed. At 1/3 sick 5 — 10 years it is also more observed any of monosyndromes — recurrent arthritis, a polyserositis, Reynaud's syndrome, Verlgofa, epileptiform, but further the disease gets a recurrent current with development of a characteristic polisindromnost.

Lupoid arthritis it is observed practically at all patients; it is shown by migrating arthralgia (see), arthritis (see), passing painful flexion contractures. Preferential small joints of brushes, radiocarpal, talocrural are surprised, large joints are more rare. At 10 — 15% of patients spindle-shaped deformation of fingers of hands, muscular atrophies on the back of brushes can develop. The joint syndrome usually is followed by mialgiya, miozita, ossalgiya and a tendovaginitis. At rentgenol, a research epiphyseal osteoporosis is found, it is preferential in joints of brushes and radiocarpal.

Fig. 1. "Butterfly" like a centrifugal erythema.
Fig. 2. "Butterfly" in the form of spots with sharp dense hypostasis.
Fig. 3. Vasculites (capillarites) on fingers.

Defeat of integuments. The syndrome of «butterfly» — erythematic rashes on a face in the field of a ridge of the nose («a body of a butterfly») and zygomas («wings of a butterfly») is most typical. According to O. L. Ivanov, V. A. Nasonova (1970), observes the following options of an erythema: 1) vascular (vaskulitny) «butterfly» — the unstable, pulsing, diffuse reddening with a cyanochroic shade in an average face zone amplifying at influence of external factors (insolation, wind, cold, etc.) or nervousness; 2) «butterfly» like a centrifugal erythema — resistant eritematoznootechny spots, sometimes with an unsharp follicular hyperkeratosis (erythema centrifugum Biett; tsvetn. fig. 1); 3) «butterfly» in the form of spots, bright pink with sharp dense hypostasis, against the background of the general puffiness and face reddening (erysipelas faciei perstans Kaposi; tsvetn. fig. 2); 4) the «butterfly» consisting of elements of diskoidny type with an accurate cicatricial atrophy. Erythematic changes are localized also on lobes of ears, a neck, a forehead, a pilar part of the head, a red border of lips, a trunk (is more often in an upper part of a thorax in the form of a decollete), extremities, over the affected joints. At a part of patients the polymorphic erythema, a small tortoiseshell, a purpura, small knots and other elements are noted.

A peculiar analog of «butterfly» of the first and second type are vasculites (capillarites) — small erythematic spots with small puffiness, teleangiectasias and an easy atrophy on trailer phalanxes of fingers of hands and legs, is more rare on palms and soles (tsvetn. fig. 3). Various trophic frustration — the hair loss, deformation and fragility of nails, ulcer defects of skin, decubituses, etc. create characteristic appearance of the patient system To. century.

Damage of mucous membranes it is shown enantemy on a hard palate, aphthous stomatitis, the milkwoman, hemorrhages, a lupoid cheilitis.

Polyserositis — the migrating bilateral pleurisy and a pericardis, is more rare peritonitis — it is considered a compound component of a diagnostic triad, along with dermatitis and arthritis. The exudate is usually small also on tsitol, reminds structure rheumatic, but contains LE cells and antinuclear factors. Recuring, polyserositis (see) leads to development of commissures up to an obliteration of a cavity of a pericardium, a pleura, an episplenitis and a perihepatitis. A wedge, manifestations of serosites usual (pains, a pericardial rub, pleurae, peritoneums etc.), but in connection with not profuseness of exudates and a tendency to bystry disappearance clinical physicians easily look through them, however at rentgenol, a research plevroperikardialny commissures or thickenings of a kostalny, interlobar, mediastinal pleura often come to light.

Lupoid carditis it is very characteristic for system To. century; it is characterized by simultaneous or consecutive development pericardis (see), myocarditis (see) or an atypical warty endocarditis of Liebman — Saks on mitral and other valves of heart, and also a pristenochny endocardium and large vessels. The endocarditis terminates in a regional sclerosis of the valve, is more rare insufficiency of the mitral valve with characteristic auskultativny symptomatology.

Defeat of vessels at system To. it is characteristic for patol century. processes in bodies. Nevertheless it should be noted a possibility of development of a syndrome of Reynaud (long before a typical picture of a disease), defeats of both small, and large arterial and venous trunks (an endarteritis, phlebitis).

Lupoid pneumonitis — vascular and connective tissue process in lungs, at an acute current proceeds as a vasculitis («vascular pneumonia»), and at other options of a current — in the form of basal pneumonitis (see) with usual a wedge, a picture of parenchymatous process, but characteristic rentgenol, the symptomatology (reticulation of the strengthened pulmonary drawing, high standing of a diaphragm and basal discal atelectases) attaches to a syndrome great diagnostic value.

Lupoid glomerulonephritis (lyupus-nephrite) — classical immunocomplex glomerulonephritis (see), it is observed at a half of patients during generalization of process as an uric syndrome, nephritic and nephrotic. The biopsy of kidneys with the subsequent gistol, and immunomorfol has great diagnostic value. research.

Defeat of the psychological sphere (neyrolyupus) — it is shown in an onset of the illness by an astenovegetativny syndrome, and at the height of a disease it is possible to observe various symptoms and syndromes from the central and peripheral nervous system, usually combined — an encephalomeningitis, entsefalopolinevrit, encephalomyelitis or meningoentsefalomiyelit with a polyradiculoneuritis (the last has diagnostic value).

At an acute form of a disease affective frustration, delirious and oneiric and delirious types of stupefaction, various on depth of a picture of devocalization can be observed.

Affective frustration are shown by conditions of an alarming depression, and also maniacal and euphoric syndromes. Alarming depressions are followed by pictures of verbal hallucinosis of the condemning contents, the sketchy ideas of the relation and nigilistic nonsense (the last differs in instability and lack of a tendency to systematization). At maniacal and euphoric states the increased mood with feeling of carelessness, content itself, total absence of consciousness of a disease is noted. Psychomotor excitement is observed from time to time a nek-swarm, persistent sleeplessness is characteristic; during the short periods of a dream — bright dreams which maintenance quite often mixes up in consciousness of the patient with real events.

Delirious and oneiric states are excessively changeable; into the forefront of frustration of a dream with fantastic or ordinary subject, plentiful colourful, stsenopodobny visual hallucinations act. Patients feel as observers of the taking place events or victims of violence. Excitement in these cases has confused and fussy character, is limited to limits of a bed, quite often is replaced by a condition of an immovability with muscular tension and loud is uniform lingering shout.

Delirious states begin with emergence during backfilling of bright dreadful dreams, in the subsequent there are multiple, color, the menacing contents visual hallucinations which are followed by verbal hallucinations, constant sensation of fear.

Intensity of mental disorders correlates with weight of somatic manifestations, with a high degree of activity of lupoid process.

The described correlations of somatopsychic frustration allow to carry psychoses at system To. century to group of exogenous organic lesions of a brain.

It must be kept in mind that at system To. century of disturbance in the emotional sphere can develop also in connection with hormonal therapy (steroid psychoses).

Defeat of reticuloendothelial system it is expressed in a polyadenia (increase in all groups limf, nodes) — very frequent and, apparently, a precursory symptom of generalization of lupoid process, and also in increase in a liver and spleen.

A current

Allocate acute, subacute and hron, disease. At the acute beginning patients can specify day of development of fever, acute polyarthritis, a serositis, «butterfly», and in the next 3 — 6 months it is possible to note the expressed polisindromnost and lyupus-nephrite or meningoentsefalomiyelit with a polyradiculoneuritis. Not treated acute system To. century earlier led to death in 1 — 2 years from an onset of the illness.

At the subacute beginning the general asthenic syndromes or recurrent arthralgias, arthritises, nonspecific damages of skin gradually develop. At each aggravation in patol, process all new bodies and systems are involved. The polisindromnost similar observed at acute disease, with a considerable frequency diffusion lyupus-nephrite and a neyrolyupus develops.

At hron, a current the disease is long is shown by a separate recurrence of these or those syndromes, and on 5 — the 10th year of a disease other organ manifestations (a pneumonitis, nephrite etc.) can join with development of a characteristic polisindromnost.

Options of the beginning and a current system To. have age patterns century. The acute current is, as a rule, observed at children and teenagers, women at climacteric age and elderly people, subacute — is preferential at women of childbearing age.

Complications

Among complications system To. century the most frequent is the consecutive infection (coccal, tubercular, fungal, virus) connected with disturbance of natural immunity either with a disease, or with inadequate treatment by corticosteroids, use of immunodepressants. At the progressing current system To. century and prolonged treatment by corticosteroid drugs, especially at young people, miliary tuberculosis therefore attention to tubercular contamination at system To develops. shall be constant for timely recognition and the corresponding correction century. Shingles (herpes zoster) develops at 10 — 15% of patients, is long treated high doses of corticosteroids and cytotoxic drugs.

The diagnosis

the Diagnosis does not represent big difficulties at patients with typical «butterfly» of any type. However this sign occurs less than at a half of patients and as a precursory symptom — only at 15 — 20% of patients. Therefore great diagnostic value is gained by other symptoms, such as arthritis, nephrite, their combinations. The possibility of an intravital biopsy of a joint and kidney allows a thicket to distinguish the lupoid nature of arthritis or nephrite. The Polisindromnost, detection of LE cells, a high caption of antinuclear factors (ANF) or antibodies to native DNA (NDNK) has diagnostic value. LE cells find at 70% of patients system To. century and more. Single LE cells can be observed also at other diseases.

Fig. 8. The immunofluorescent test for detection of an antinuclear factor anti-scale the globulinovy serum conjugated by fluorescein thiocyanate (an indirect immunofluorescence, substrate — a cut of a rat liver): 1 — a peripheral luminescence; 2 — a homogeneous luminescence; 3 — a speckled luminescence; 4 — a nukleolyarny luminescence; x 270.

ANF is IgG directed against kernels of cells of the patient. Usually apply the Immunofluorescent method (fig. 8) to definition of ANF, at the same time as antigenic material take cuts of a rat liver rich with kernels on which layer serum of the patient and marked flyuorestseiny anti-globulins. For system To. century the peripheral, regional luminescence (fig. 8,2) caused by existence of antibodies to DNA and a high caption of this reaction is most characteristic.

Antibodies to DNA are defined by various methods in THRESHING BARN (see. Hemagglutination ), in a cut mutton erythrocytes are loaded with DNA, in a flocculation test of particles of bentonite (see. Flocculation ), also loaded DNA; besides, use a method of radio immune binding marked on NDNK iodine and an immunoflyuorestseniiya where as NDNK substrate take culture of Crithidia luciliae.

At hron, polyarthritis and severe damage of a liver positive reactions to a rhematoid factor in Voler's reaction — Rouza can be found (see. Pseudorheumatism ) or latex agglutination (see. Agglutination ). Also the research of a complement CH50 and its components which decrease usually correlates with activity of lupoid nephrite is useful. Almost at all patients ROE — to 60 — 70 mm an hour is considerably accelerated. More than at a half of patients the leukopenia (lower than 4000 in 1 mkl) with shift in a blood count to promyelocytes, myelocytes and young in combination with a lymphopenia (5 — 10% of lymphocytes) is observed. Moderate hypochromia anemia is very often observed. In rare instances hemolitic anemia with lines of acquired develops hemolysis (see) and positive reaction of Koombs (see. Koombs reaction ). Thrombocytopenia (lower than 100 000 in 1 mkl) is observed often, in rare instances — Verlgof's syndrome.

Thus, at establishment of the diagnosis system To. it is necessary to consider all a wedge, a picture, data a lab century. methods of a research and biopsy material of kidneys, synovia and skin.

For more omnibus estimate of a condition of the patient it is reasonable to define a degree of activity patol, process. Wedge, and lab. the characteristic of degrees of activity system To. it is given in table 1 century.

Treatment

the Treatment begun in an onset of the illness gives the best effect. To the acute period treatment is carried out in a hospital where patients should provide good nutrition with enough vitamins of group B and C.

For individualization of treatment crucial importance has the differentiated definition of degrees of activity patol, process (tab. 1).

At patol, process of the III degree of activity by all patient, irrespective of option of a current, treatment by glucocorticosteroids in high doses (in day of 40 — 60 mg of Prednisolonum or other drug in equivalent doses) is shown, at the II degree — respectively smaller doses (30 — 40 mg a day), and at the I degree — 15 — 20 mg a day. It is extremely important that the initial dose of glucocorticosteroids was sufficient for reliable suppression of activity patol, process. It is necessary to appoint especially high doses (50 — 60 — 80 mg in day of Prednisolonum) at a nephrotic syndrome, an encephalomeningitis and other diffusion processes in a nervous system — so-called lupoid crisis. Treatment by glucocorticosteroids in the maximum dose is carried out to approach of the expressed effect (according to decrease in clinical laboratory indicators of activity), and at a nephrotic syndrome — not less than 2 — 3 months, then the dose of hormone is slowly reduced, being guided by the offered scheme (tab. 2), but observing the principle of individualization for the purpose of prevention of a withdrawal or a syndrome of a dose decline.

It is necessary to appoint glucocorticosteroids in a complex with drugs of potassium, vitamins, anabolic hormones and symptomatic means (diuretic, hypotensive, ATP, cocarboxylase, etc.). At decrease in their dose it is necessary to add salicylates, aminoquinolinic and other drugs. Treatment by hormones, as a rule, does not manage to be cancelled completely in connection with quickly developing aggravation of symptoms (withdrawal) therefore it is important that the maintenance dose was minimum. A maintenance dose usually 5 — 10 mg of drug, but maybe higher at unstable remission.

Such collateral symptoms arising against the background of treatment as cushingoid, a hirsutism, ecchymomas, striya, acnes, develop at many patients and additional therapy do not demand. On the contrary, it is noted that permanent improvement of a state usually occurs at development of signs of overdose of hormones. At persistent hypostases diuretics, plasma transfusions, albumine can be recommended. Hypertensia is rather easily stopped by hypotensive drugs.

Such complications as steroid ulcer, aggravations of focal infection, disturbance of mineral metabolism with osteoporosis, etc. are much more serious, but also they can be prevented at systematic control. An undoubted contraindication to continuation of treatment is steroid psychosis or strengthening of convulsive attacks (epilepsy). Correction by psychotropic drugs is necessary.

At inefficiency of glucocorticosteroids the patient system To. appoint treatment cytostatics immunodepressants of the alkylating row (cyclophosphamide) or metabolites (Azathioprinum) century. Indications to purpose of these drugs at system To. are century: a high (III) degree of activity of a disease with involvement in process of many bodies and systems, especially at teenagers; the developed lupoid nephrite (nephrotic and nephritic syndromes); need to reduce an overwhelming dose of a glucocorticosteroid because of the developed by-effects of this therapy.

Azathioprinum (imuran) and cyclophosphamide appoint in doses 1 — 3 mg to 1 kg of weight of the patient a day in combination with 10 — 40 mg of Prednisolonum a day for control of extrarenal symptoms. Treatment by immunodepressants also shall be long on condition of regular observation of the doctor. At treatment by immunodepressants serious complications therefore control of blood (including thrombocytes) and urine, especially in the first 3 weeks of treatment is necessary can develop. At inf. complications an active antibioticotherapia is made. Other complications, including a total alopecia, pass at reduction of a dose of an immunosuppressant and purpose of symptomatic therapy.

At hron, a current system To. century with preferential damage of skin as diskoidny To. recommend chloroquine, delagil or other drugs of a quinolinic row century.

Pr both subsiding of signs of damage of internals and decrease in clinical laboratory signs of activity to the I degree it is possible to apply to lay down. physical culture and massage under control of the general state and condition of internals. A physiotherapeutic and resort therapy at system To. it is not recommended because of a possibility of provocation of a disease by uv radiation, a balneoterapiya, insolation century.

The forecast

the Forecast for life at early recognition system To. century and adequate to activity patol, process prolonged treatment satisfactory; 70 — 75% of patients are returned to active work on production and in a family. However at development of lyupus-nephrite, a tserebrovaskulit and accession of consecutive infection the forecast worsens.

Prevention

Prevention is directed to prevention of aggravations and progressing of a disease and developing of a disease.

Prevention of progressing of a disease (secondary) is performed by timely adequate, rational complex therapy therefore patients shall undergo regularly dispensary inspection, accept hormonal drugs in strictly appointed dose, not sunbathe and not overcool, avoid operative measures, inoculations of vaccines and serums (except vital). At an exacerbation of a focal or intercurrent infection the bed rest, antibiotics, the desensibilizing therapy is obligatory. Treatment of focal infection shall be persistent, preferential conservative.

Measures of primary prevention are especially important at members of families of patients system To. century, having signs of a photosensitization, intolerance of drugs, disturbances of humoral immunity. For prevention of a disease or generalization of process these people should avoid uv radiation, treatment by radio isotope gold, a resort therapy etc.

Features of a current of a system lupus erythematosus at children

Are surprised preferential girls of prepubertatny and pubertal age. Rise in incidence begins from 9th year of life, its peak falls on 12 — 14 years. Sometimes system To. occurs at children of 5 — 7 years century; as casuistic cases of children of the first months of life are described. Cases of an inborn disease do not happen.

In most cases at children and teenagers system To. begins century and proceeds more sharply and heavier, giving higher mortality, than at adults. It is caused by features of reactivity of the growing organism, an originality of connective tissue structures, bodies of an immunogenesis, system of a complement, etc. Generalization patol, process at children develops much quicker, and defeat of various bodies is characterized by dominance of an exudative component of an inflammation in combination with signs of intensively developing syndrome of intravascular disturbance of a blood coagulation in the form of hemorrhages and bleedings, kollaptoidny, soporous and depressed cases, thrombocytopenia.

In an onset of the illness most often children show complaints to joint pains, weakness, an indisposition. Along with it fever is noted, dystrophy quickly enough accrues, edges reaches quite often a cachexia, considerable changes from blood appear, signs of damage of many vitals and systems come to light.

Changes of skin in manifestations, typical for a lupus, meet not always. The combination of acute exudative and diskoidny changes, and also tendency to merge of the separate centers of defeat to total spread of the dermatitis taking all integument and a pilar part of the head is characteristic. Hair strenuously drop out that leads to a gnezdny alopecia or full baldness, break off, forming a peculiar brush over the line of a forehead. Mucous membranes of a mouth, upper respiratory tracts, generative organs can be surprised. Nonspecific allergic manifestations in the form of a small tortoiseshell and korepodobny rash or the mesh and vascular drawing of skin, and also petekhialno-hemorrhagic elements meet considerably more often and can be found almost in each patient in the active period system To. century.

The joint syndrome which is the most frequent and practically always one of the first symptoms of a disease can be presented by arthralgias of flying character, acute or subacute arthritis and a periarthritis with easy ephemeral exudative manifestations. The joint syndrome is usually combined with defeat of the tendinous and muscular device though mialgiya and miozita sometimes are an independent sign system To. century.

Involvement in patol, process of serous covers is observed practically in all cases; in clinic pleurisy and a pericardis, usually in combination with a perihepatitis, an episplenitis, peritonitis is most often distinguished. The massive exudate in a pleura, the pericardium demanding repeated punctures is characteristic manifestations system To. century.

One of the most frequent visceral signs system To. century the carditis is; its combination to arthritis at early stages of a disease is almost always mistakenly treated as rheumatism. All three covers of heart can be surprised, but at children and teenagers symptoms of myocarditis prevail.

Damage of lungs in clinic is defined less than damage of a pleura. The typical lupoid pneumonitis is followed by the alveolar and capillary block, and percussion auskultativnye data scanty, however the accruing hypoxia, the phenomena of respiratory insufficiency attract attention, confirm existence of a pneumonitis and rentgenol, data.

Lupoid nephrite occurs at children and teenagers more often than at adults (approximately in 2/3 cases) and at the vast majority of patients represents severe damage of kidneys with a nephrotic syndrome, a hamaturia, tendency to arterial hypertension, often is followed by an eclampsia. On character of a current lyupus-nephrite at children is close to the mixed form hron, a banal glomerulonephritis, quite often it represents option of quickly progressing glomerulonephritis and only at some patients proceeds in the form of the minimum uric syndrome.

The defeat of the central and peripheral nervous system generally similar to that at adults, includes also a choreoid syndrome with all a wedge, the lines inherent in small to a chorea (see).

Quite often signs of defeat meet went. - kish. path. Abdominal pains can be caused by damage of intestines, development of peritonitis, an episplenitis, perihepatitis, and also hepatitis, pancreatitis. Before establishment of the diagnosis system To. century abdominal crises can be taken for a banal acute appendicitis, cholecystitis, ulcer colitis, dysentery, etc. Sometimes the picture develops acute abdomen (see). The symptom complex zlokachestvenno of the current disease Krone is possible. The active period of a disease is followed by increase peripheral limf, nodes, sometimes so considerable that for differential diagnosis their puncture or a biopsy is required.

At 2/3 sick children and teenagers system To. develops sharply or subacutely century; also cases of the acute disease can be observed, for to-rogo rapid development of giperergichesky reactions, high fever of the wrong type and other signs (damage of skin, joints, limf, nodes), hemorrhagic diathesis, defeat of a nervous system is characteristic. Quickly progressing vasculitis in a short space of time leads to heavy vospalitelnodestruktivny and dystrophic changes of internals (heart, kidneys, lungs), with disturbance of their functions and a possible lethal outcome in the first 3 — 9 months from the beginning of a disease. Death in such cases most often comes at the phenomena of a cardiopulmonary and (or) renal failure against the background of intoxication, deep disturbances of a homeostasis, koagulopatichesky disturbances, a water and electrolytic imbalance, and also accession of consecutive infection.

At subacute system To. century, average on weight and duration, generalization of process comes in the first 3 — 6 months from the beginning of a disease, a current persistent or wavy with constantly remaining signs of activity and rather quickly joining funkts. inferiority of this or that body.

Approximately at 1/3 children the option of primary and chronic disease close to a picture classical system is observed To. century of adults, with the presystem period lasting from one to 3 years and with the subsequent generalization of process. Among presystem lupoid manifestations at children gemopatiya, hemorrhagic and nephritic syndromes, arthropathies, a chorea most often act. Also other more rare monosyndromes are possible.

Complications and diagnostic methods same, as at adults.

Each child with the expressed clinical laboratory signs of activity system To. it is necessary to treat in the conditions of a hospital century. For suppression of an immune hyperactivity use corticosteroids and cytostatics. The size of their daily dose is defined not only by age of the child, but also a degree of activity patol, process. At activity of the III degree with symptoms of nephrite, a carditis, a serositis, a neyrolyupus appoint high doses of corticosteroids (Prednisolonum at the rate of 1,25 — 2 mg and more to 1 kg of weight of the patient a day). If the patient cannot give the specified dose of Prednisolonum or equivalent amount of similar drug, it is necessary to enter Azathioprinum or cyclophosphamide at the rate of not less than 1 — 3 mg on 1 kg a day into therapy. At a nephrotic syndrome, autoimmune hemolitic anemia, a hemorrhagic syndrome and krizovy states in all cases the combined immunodepressive therapy in combination with heparin is from the very beginning carried out (250 — 600 PIECES on 1 kg of weight a day). On reaching clear clinical laboratory improvement in a condition of the patient the maximum immunooverwhelming dose of Prednisolonum it is necessary to reduce (tab. 2), heparin is replaced with antiagregant (curantyl) and (or) anticoagulants of indirect action.

At a moderate degree of activity system To. century the immunooverwhelming dose of corticosteroids shall be lower (Prednisolonum — 0,5 — 1,2 mg on 1 kg of weight a day), instead of heparin appoint curantyl on 6 — 8 mg to 1 kg of weight a day, use salicylates, drugs of a quinolinic row, a metindol more widely. At hron, a current and a low degree of activity system To. century with lack of clear symptoms of damage of kidneys, blood, a nervous system, heart, lungs corticosteroids appoint in small doses (Prednisolonum — less than 0,5 mg to 1 kg of weight a day) or do not use at all.

After an extract from a hospital children are under observation of the rheumatologist and continue to receive the supporting immunodepressive and symptomatic therapy. For the first year after the acute period system To. century the school is not recommended to be attended, but it is possible to organize training at home. It is necessary to cancel all planned preventive inoculations.

At adequate treatment of patients even more often it is possible to achieve relative or full remission. At the same time the general physical. development of children goes more or less well, secondary sexual characteristics timely appear, at girls the periods begin in time. The lethality is most often connected with a renal failure.

The Diskoidny lupus erythematosus

Diskoidnaya To. century (synonym: lupus erythematodes discoides s. chronicus, erythematodes, seborrhea congestiva, erythema atrophicans etc.) — the most frequent chronic form K. century, at a cut damage of skin and mucous membranes is dominating in a picture of a disease. The name «lupus erythematodes» was offered by P. Kazenav in 1851, believing that the disease is a kind of a tubercular lupus. For the first time it was described by Raye (R. of F. Rayer) in 1827 as a rare form of the grease expiration (fluxus sebaceus). Diskoidny To. makes 0,25 — 1% of all dermatosis century (M. A. Agronik, etc.), meets in the countries with a frigid humid climate more often, it is preferential at middle-aged persons [W. Gertler]. Women are ill more often than men.

The etiology

the Aetiology is not finalized. The virus origin of a disease is supposed. At a submicroscopy find tubuloretikulyarny cytoplasmic inclusions in the centers of damage of skin.

A pathogeny

In a pathogeny of separate cases factors matter genetic and immunol. In provoking diskoidny To. century and its aggravations an important role is played by excess insolation, medicines, different injuries (mechanical, thermal, chemical).

Pathological anatomy

Fig. 9. Microscopic changes of skin in the centers of defeat at a diskoidny lupus erythematosus: 1 — a hyperkeratosis; 2 — a White's disease; 3 — perivascular cellular infiltrate, according to D. L. Gardner.

Diskoidny To. century and its disseminated form is limited to skin changes. At diskoidny To. century defeat is localized more often on a face. Microscopically (fig. 9) find hyperkeratosis (see), a White's disease, vacuolar dystrophy of epidermis (see. Vacuolar dystrophy ), acanthosis (see). In a derma focal lymphoid and macrophagic infiltrates with impurity of neutrophils, plasmocytes are visible. Walls of blood vessels are impregnated with plasma proteins. The collagenic fibers of a derma which bulked up, pikrinofilny, merge in fibrinoid masses. In a zone of infiltrates elastic and collagenic fibers are destroyed. During treatment there is a scarring to an atrophy and depigmentatsiy skin.

For the disseminated skin form K. century multiple rashes on all body in which microscopic changes remind those at diskoidny To are characteristic. century, however are expressed more weakly, exudative reactions prevail over proliferative, cellular infiltration is less considerable. In the outcome there are no hem left and sites of an atrophy of skin.

Clinical picture

Fig. 4. It is long the existing center of a diskoidny lupus erythematosus on a cheek of the patient: atrophic and erythematic zones are expressed.

Diskoidny To. begins century with emergence of one-two pink slightly edematous spots which gradually increase in a size, infiltrirutsya, become covered in the central area by densely sitting whitish scales. Poskablivaniye of the centers hurts (Benye's symptom — Meshchersky) since on the lower party of a scale there is a horn spinule (a symptom of a ladies' heel), the Crimea it is strengthened in the expanded mouth of a hair follicle. Further in the central part of the center the cicatricial atrophy develops. In it is long the existing center clearly distinguish three zones: central atrophic, further giperkeratotichesky and bordering it erythematic (tsvetn. fig. 4). Within the last often are available teleangiectasias (see). On the periphery of the center in different degree the brown hyperpegmentation can be expressed. Erythema (see), hyperkeratosis and atrophy of skin (see) — cardinal symptoms To. century. Infiltration, teleangiectasias and pigmentation are frequent, but not obligatory signs.

Fig. 5. Lupoid "butterfly" on a face of the patient with diskoidny damage of skin.

The most characteristic localization diskoidny To. century — sites the skin subject to insolation: person, hl. obr. its middle part — Nov, cheeks, malar, preear areas. As well as for system To. century, the so-called butterfly is very characteristic (tsvetn. fig. 5) — the center of defeat on a ridge of the nose and cheeks. By data I. I. Lelis observing 518 patients, primary centers To. were located on a nose at 48%, on cheeks century — at 33%, on auricles or the skin adjoining to them — at 22,5%, on a forehead — at 16,5%, on a pilar part of the head — at 10%, on a red border of lips, usually lower — at 12,5%, on a mucous membrane of a mouth — at 7%. Damage of a mucous membrane a century L. I. Mashkilleyson with sotr. observed at 3,4% of patients. More rare are known, including the isolated localizations — on a breast, a back, shoulders, etc. Damages of a mucous membrane of genitalias, a bladder, a cornea, damage of nails are described. Along with typical diskoidny To. there are its versions century: giperkeratotichesky To. century, at a cut the hyperkeratosis is sharply expressed; papillomatous diskoidny To. century — the strengthened growth of nipples of a derma leading to formation of a fleecy surface of the centers; warty To. century — the papillomatosis is followed by strong keratinization; pigmental To. century — excess adjournment of the pigment painting the centers of defeat in dark brown color; seborrheal To. century — hair follicles are strongly expanded and filled with fat friable scales; tumorous To. century — the cyanotic-red strongly towering centers with the edematous accurately outlined edges, a slight hyperkeratosis and an atrophy.

Rare versions are telangiectatic diskoidny To. century with multiple teleangiectasias, hemorrhagic diskoidny To. century with hemorrhages in the centers, mutiliruyushchy. Special form hron. To. century the centrifugal erythema (erythema centrifugum Biett) is. It makes 5,2 — 11% in relation to all forms K. century, it is characterized by accurately delimited centers of an erythema on a face, is more rare on other sites of skin. In them there can be teleangiectasias, small puffiness. There is no hyperkeratosis. The atrophy is absent or is expressed poorly. The centrifugal erythema quickly enough concedes to treatment, but easily recurs. Some authors rank it, along with disseminated To. century, to forms, intermediate between diskoidny and system.

In the centers diskoidny To. century on a mucous membrane of a mouth a dark red erythema, teleangiectasias, polosovidny, rough setevidny sites of opacification of an epithelium, an erosion, superficial ulcerations are observed. On a red border of lips To. has an appearance of incorrectly oval tape-like centers of an erythema and a hyperkeratosis, sometimes with cracks, erosion century. The centers diskoidny To. century single are more often, multiple are more rare. Without treatment they exist for years, as a rule, do not cause unpleasant feelings. Erosive and ulcer rashes in a mouth cause morbidity. Especially persistently they proceed at smokers. Disseminated diskoidny To. it is characterized by scattered erythematic and edematous, papular elements or the centers of diskoidny type century. Preferential localization: the person, an open part of a breast and back, a brush, foot, skin over elbow and knee joints. The general condition of patients diskoidny and disseminated To. century, as a rule, considerably does not suffer. However at a wedge, inspection at 20 — 50% of patients arthralgias, funkts, disturbances come to light from internals (heart, a stomach, kidneys), a nervous system, the accelerated ROE, a leukopenia, hypochromia anemia, changes in composition of immunoglobulins, antinuclear antibodies, cell-bound immune complexes in a zone of dermoepidermalny connection, etc.

Deep To. century (L. e. profundus Kaposi — Irgang) is characterized by simultaneous existence of the typical centers of damage of skin characteristic for diskoidny To. century, and nodes in hypodermic cellulose, skin over to-rymi mostly is not changed. Number of authors, e.g. Potriye (L. M of Pautrier), consider this form as a combination of deep sarcoids to Darya — Russia and diskoidny To. century

Complications

the carcinoma cutaneum Occasionally develops, it is preferential in the centers on a red border of an under lip, is very rare — sarcoma, an erysipelatous inflammation; the heavy complication which more often is observed at disseminated diskoidny To. century, its transition in system is To. century under the influence of adverse factors.

The diagnosis

the Diagnosis in typical cases is established without difficulties. The centers diskoidny To. can have looking alike seborrheal eczema, pink eels, psoriasis, an eosinophilic granuloma of the person, a tubercular lupus century. The clear boundary of the centers, horn traffic jams in expanded hair funnels which are densely sitting scales, a positive symptom of Benye — Meshchersky, development of an atrophy demonstrate existence To. century. Centers of seborrheal eczemas (see) have no so sharp borders, their surface is covered with friable fat scales, they well give in to antiseborrheal therapy. The psoriasis centers are usually numerous, covered with easily scratched out silvery scales (see. Psoriasis ). Both that and others in contrast To. century usually decrease under the influence of sunshine. At pink eels (see) there is a diffuse erythema, teleangiectasias are sharply expressed, small knots and pustules often appear. Eosinophilic granuloma of the person (see) it is characterized by special persistence to therapeutic influences. The centers it more often single, uniform brown-red color, without hyperkeratosis, with single teleangiectasias. A tubercular lupus (see. Tuberculosis cutis ) usually begins with the childhood, existence lyupy with phenomena of apple jelly and the probe inherent to them is characteristic of it. In cases of an erythematic tubercular lupus erythematosus of Leluar the wedge, the diagnosis is extremely difficult, the research is necessary gistol. Diskoidny To. it is necessary to differentiate also with lymphocytic infiltration of Essner century — Kanoffa, manifestations a cut differ in smaller firmness, tendency to permission in the center, lack of a peeling, hyperkeratosis, atrophy. To. century on a pilar part of the head differentiate with pseudopelade (see). Lack of an inflammation, horn spinules, finger-shaped arrangement, more superficial atrophy is characteristic of the last. Diskoidny To. century on a mucous membrane of a mouth it is necessary to distinguish with red flat it is deprived, rashes to-rogo have more gentle drawing, are not followed by an atrophy.

Patients diskoidny To. century, including and limited forms, shall be inspected for the purpose of an exception of systemic lesion of internals and a nervous system, and also detection of associated diseases.

Treatment

the Leading role in therapy diskoidny and disseminated To. belongs to aminoquinolinic drugs century — to chloroquine, Resochinum, delagil at, plaquenil at, etc. They are appointed continuously or cycles usually on 0,25 g by 2 times, plaquenil — on 0,2 g 3 times a day after food. Duration of cycles (5 — 10 days) and intervals between them (2 — 5 days) depends on portability of treatment. Repeated courses of treatment, especially in spring time are recommended. Accession to chloroquine of small doses of corticosteroids (2 — 3 tablets of Prednisolonum a day) improves results of treatment and portability. This technique is recommended at especially persistent current To. century, extensive damage of skin.

It is useful to include B6, V12 vitamins, pantothenate of calcium in a therapeutic complex, nicotinic to - that. Lech. the effect occurs quicker at co-administration of ointments with fluorinated corticosteroids (Synalarum, flyutsinar, etc.) which at the limited centers can be also the main method of therapy. Also intradermal introduction to poryazhenny sites of 5% of solution of chloroquine of 1 times in 5 — 7 days is recommended (4 — 6 obkalyvaniye on a course). The limited centers with powerful infiltrate and a hyperkeratosis without signs of peripheral growth can be subjected to cryotherapy.

The forecast

the Forecast for life favorable. At adequate treatment, observance by patients of the recommended mode working capacity them remains for many years.

Prevention

Patients To. century medical examinations are subject. They shall observe a gigabyte. the mode of work, rest, food to avoid physical. and nervous overloads, stay in the sun, wind, a frost to apply photoprotective creams and films with paraaminobenzoic to - that, a tannin, etc. It is necessary to sanify the centers of a focal infection. For treatment of associated diseases of patients To. it is not necessary to direct on yuzh century. resorts in spring and summer time, they should appoint physiotherapeutic procedures with care, to vaccinate only at serious indications.

A medicinal lupus erythematosus

Medicinal To. develops in connection with long reception of apressine (gidralazin), novokainamid (procaineamide), dipheninum (hydantoin), Trimethinum (Trimethadionum), carboazepine, an isoniazid and Chlorpromazinum century. Medicinal To. can develop at the elderly people having hypertensia and arrhythmia at TB patients and epilepsy century. The listed drugs are capable to cause formation of anti-nuclear antibodies (ANF, antibodies to DNA) which emergence precedes clinic medicinal To. century, reminding system To. century. At reception of some drugs there is a certain wedge, a syndrome. So, at apressinovy To. century the glomerulonephritis develops, at long reception of niacinamide the pleurisy and pneumonites which are the beginning of a syndrome are very frequent.

Among mechanisms there is development medicinal To. century the role of predisposition as such reaction arises approximately at 10% of the patients accepting apressine and other drugs, and also disturbances of metabolism, in particular speeds of acetylation of these drugs is discussed.

The diagnosis is made on the basis of reception of the listed drugs.

Timely recognition of a disease and cancellation of the medicine causing medicinal To. century, leads to recovery, however there can be a need of purpose of corticosteroids for average doses (20 — 30 mg of Prednisolonum a day), especially at izoniazidny medicinal To. century. At development of clinic system To. century the corresponding therapeutic tactics is necessary.

See also Autoallergichesky diseases , Collagenic diseases .


Tables

Table 1. Clinical and laboratory exponents of activity of a system lupus erythematosus


Table 2. The approximate scheme of reduction of a dose of Prednisolonum depending on an initial (maximum) dose




Bibliography: Vinogradova O. M. A system lupus erythematosus in clinic of internal diseases, Owls. medical, No. 4, page 15, 1958; At with e in and L. L. and JI at N and nanosecond to and I am I. R. Psychopathological manifestations at a system lupus erythematosus, Zhurn, a neuropath, and psikhiat., t. 75, century 4, page 562, 1975, bibliogr.; Davydovsky I. V. To a question of lupus erythematodes disseminatus acutus, Russian vestn. dermas., t. 7, No. 5, page 450, 1929, bibliogr.; And T. I. smiles and F r at m to and - N and S. L. K of psychopathology and to a pathogeny of symptomatic psychosis at a system lupus erythematosus, Zhurn, a neuropath, and psikhiat., t. 72, No. 12, page 1860, 1972; L of e of l and with I. I. Lupus erythematosus, L., 1970, bibliogr.; Meshchersky G. I. and Grinchar F. N. About a case of erythema faciei perstans (Kaposi — Kreibich’a) of a tubercular origin, Harkovsk. patologoanat. Saturday., it is devoted. to the prof. M. N. Nikiforov, to the 25 anniversary its Wuchang, deyateln., page 406, M., 1911; Nasonova V. A. System lupus erythematosus, M., 1972, bibliogr.; With e r about in V. V. and d river. The immunomorphological characteristic of changes of skin at a lupus erythematosus, Soz. medical, No. 9, page 15, 1972; With e r about in V. V. and d river the Elektronnomikroskopichesky characteristic of lupoid nephrite, Arkh. patol., t. 36, No. 6, page 21, 1974, bibliogr.; With to r and p to and N Yu. K., Somov B. A. and B at t about in Yu. S. Allergic dermatosis, page 130, M., 1975, bibliogr.; With t r at-to about in A. I. and B of e of of l and r I am A. G N. Pathological anatomy and pathogeny of collagenic diseases, page 248, M., 1963; To Tara-e in E. M. Collagenoses, M., 1965, bibliogr.; Tareeva I. E. Lupoid nephrite, M., 1976, bibliogr.; Tareeva I. E., Serov V. V. and Kupriyanova L. A. Intra endothelial inclusions at a system lupus erythematosus, Bulletin Ekspery, biol, and medical, t. 77, No. 5, page 119, 1974; About’ With about n n about r J. F. and. Musher D. M of Central nervous system involvement in systemic lupus erythematosus, Arch. Neurol. (Chic.), v. 14, p. 157, 1966; Hargraves M. M., Richmond H. a. M o r t o n R. Presentation of two bone marrow elements, «tart» cell and the «L. E.» cell, Proc. Mayo Clin., v. 23, p. 25, 1948; Klemperer P., Pollack A. D. a. Baehr G. Pathology of disseminated lupus erythematosus, Arch. Path., v. 32, p. 569, 1941; Lupus erythematosus, ed. by E. L. Dubois, Los Angeles, 1974; Recent advances in rheumatology, ed. by W. W. Buchanan a. W. C. Dick, pt 1, Edinburgh — L., 1976; Ropes M. W. Systemic lupus erythematosus, Cambridge — L., 1976, bibliogr.


V. A. Nasonova; L. A. Isaeva (ped.), A. I. Strukov, L. V. Kaktursky (stalemate. An.), A. S. Tygans (psikhiat.), L. Ya. Trofimova (dermas.).

Яндекс.Метрика