ShYoNLEYNA — GYONOH the DISEASE
(J. L. Schonlein, is mute. therapist, 1793 — 1864; E. H. Henoch, is mute. pediatrician, 1820 — 1910; synonym; an abdominal purpura, an allergic purpura, a hemorrhagic vasculitis, Genokh a hemorrhagic purpura, a capillary toxicosis, a microthrombovasculitis hemorrhagic, a purpura kapillyaropatichesky, Shenleyna — Genokh an anaphylactoid purpura) — the aseptic inflammation of small vessels caused by the damaging action of cell-bound immune complexes and which is shown hemorrhages, disturbance of intravascular coagulability of blood and a mikrotsirkulyatorny-ma frustration. The term «Shenleyna — Genokh a Disease» is not standard, abroad before -
the nochteniye is given to the term «anaphylactoid purpura». In the USSR, but to V. A. Nasonova's (1959) proposal, the disease is called more often a hemorrhagic vasculitis. 3. S. Barkagan (1980) considers that he captures the essence patol more precisely. process term «microthrombovasculitis».
The disease is for the first time described in 1808. In illany, to-ry noted that hemorrhages on skin can be combined with abdominal pains, a melena, hypostases. I. Shenleyn in 1837 paid attention to frequent damage of joints at patients with hemorrhagic rashes and designated this form of a disease as «peliosis rheu-matica». The option of a disease, at Krom in clinic dominates a picture of an acute abdomen and (or) went. - kish. bleedings, it was in details characterized in 1868 and 1874 by Genokh. It designated it as «purpura abdominalis». The accompanying damage of kidneys at patients with a hemorrhagic purpura was described in 1852 by G. Johnson, however the early systematized studies of damage of kidneys belong to Genokh (1899) and U. Os-leru (1914). In 1914 Osler for the first time connected a pathogeny of a hemorrhagic purpura with an anaphylaxis (see). This representation was developed in the subsequent by the researches Glantsman-na (E. Hlanzmann, 1920). Typical gistomorfol. signs of damage of skin at this disease are described in 1890 by Zilbermannom (O. Sil-bermann) and in 1900. U. Os lery. The wedge, a picture of a generalized vasculitis with the detailed characteristic of each symptom were described in 1940. A. N. Kryukov and K. I. Agama fishing, having emphasized an important role of an infection in its development.
Highway — G. meets at children's age more often. By data A. V. Pa-payana and N. P. Shabalov (1982), it is registered with
a frequency of 23 — 26 on 10 000 faces aged to 14 years. Incidence increases in the early spring and in the fall. At adults more often than at children, symptomatic vasculites as display of limfoproliferativny diseases, hron are observed. active hepatitis, tumors, pseudorheumatism, tuberculosis, system lupus erythematosus, bacterial endocarditis.
Etiology. The disease can be carried to iolietiologichesky since it is supposed that the antigen which is a part of the cell-bound immune complexes damaging vessels can be the different nature. It is known that III. —. often bacterial or viral infections, inoculations, reception of medicines, the use of the products causing an allergy precede; there is also an instruction on possible communication of a course of a disease with a parasitic invasion, overcooling, excess insolation, an injury, contact with insecticides. A part is played, apparently, also by genetically determined features of immunity; in cases when the disease gets hron. a current, autoimmune mechanisms matter.
The pathogeny is completely not opened. Major importance is attached to the damaging action on vessels of low-molecular complexes antigen — an antibody (see Antigen — an antibody reaction), to-rye owing to defective reactions of immunity begin to circulate in blood or to pretsipitirovat in small vessels. Sticking of cell-bound immune complexes to a surface of endothelial cells leads to their damage, activation of system of a complement (see) and systems of a blood coagulation (see. Coagulant system of blood). The hemotaksisny factors attracting in the center of damage the neutrophils englobing mononukleara, basophiles, eosinophils, mast cells that creates a picture of an aseptic inflammation are formed. In the course of phagocytosis of a cell degranulate, releasing procoagulant factors, vasoactive amines (a histamine, serotonin); at the same time the kallikrein-kinin system is activated (see Kinina). As a part of the granular deposits found around vessels reveal immunoglobulins of a class A, SZS, C3d, C5 (see Immunoglobulins). Immunoglobulins of a class M, S4, SZ are registered less often, Clq and SZ-proaktivator (a factor In) do not come to light in general that can testify to preferential complement activation at III. —. on an alternative way.
As a result of activation of fermental systems vessels extend, their permeability increases, the basal membrane becomes bare, and cell-bound immune complexes get into an intima of a vessel, and also into interstitial substance. Thrombocytes after contact with collagen of a basal membrane are activated, releasing III and IV factors of coagulation; also Hageman's factor is activated (see. Hemorrhagic diathesis). The disseminated blood coagulation causes blockade of a mikrotsir-kulyatorny bed that aggravates dystrophy of vessels and their aseptic inflammation. At long circulation in blood of cell-bound immune complexes of change in walls of vessels reach degree of a necrosis, become irreversible that leads in some cases to considerable defeat of fabrics and bodies, in to-rykh it is localized patol. process (skin, joints, kidneys, peritoneum, mesentery, sometimes vessels of lungs and brain). At an inflammation of vasa vasorum thrombophlebitises are possible, to-rye can be complicated by a thromboembolism (see).
Pathological anatomy. At gistol. a research find signs of an aseptic inflammation of vessels with preferential defeat of a microcirculator bed — arterioles, capillaries, venules. At a research of affected areas of skin reveal signs of a fibrinoid necrosis, platelet blood clots, adjournment in walls of vessels and an interstitium of mass of fibrin. In perivascular space neutrophils, in nek-ry cases — eosinophils are often visible. Interstitial fabric is edematous, contains erythrocytes. Changes in kidneys are more often characterized by a picture of focal, is more rare than a diffusion glomerulonephritis (see). Focal proliferation of preferential mezangi-alny cells of balls, increase in a mezangialny matrix is noted, epithelial semilunums are typical. Changes of vessels of kidneys are rather rare. Sometimes the thickening of an intima, inflammatory changes, a fibrinoid necrosis, obstruction of vessels platelet blood clots meet. At fluorescent microscopy most constantly find deposits of IgA, SZ, fibrin, is more rare — deposits of IgG. Deposits are located preferential in a mezangiya, but not along a basal membrane. During the involvement in patol. process of vessels of a mucous membrane went. - kish. a path in it find the hemorrhages merging among themselves, sites of a necrosis, ulcer defects. Gistol. the picture is same, as well as in affected areas of skin. Changes in lungs are most often limited to a focal productive or exudative alveolitis, the basis to-rogo is made by capillarites of interalveolar partitions.
Clinical picture. Distinguish the following forms Sh. — G.: the acute, including fulminant purpura differing in a heavy current up to a lethal outcome in several days after the beginning of a disease; chronic recurrent, for a cut alternation of remissions with a recurrence is characteristic. Consideration of a fulminant purpura as forms of a current of Sh. — G. remains disputable, despite a pathogenetic community of trombogemorragichesky frustration. At children in 50 — 90% of cases, and at adults approximately in 30% of cases the disease is preceded by a respiratory infection (for 1 — 3 week till its beginning). The first manifestations of Sh. — G. can be not specific (an indisposition, headaches, fever, a polyarthralgia, abdominal pains). The acute form of a disease in most cases begins suddenly with emergence and distribution of a hemorrhagic enanthesis of the lower extremities, buttocks, side surfaces of a stomach, in some cases — with sharp, colicy pains in a stomach as colic, a bloody diarrhea, a melena, vomiting with impurity of blood, sometimes in a wedge, to a picture polyarthritis dominates (as rheumatic).
ShENLEYNA-GENOHA the DISEASE of 399
Fulminant purples is observed preferential at children of preschool age after the postponed viral diseases (measles, a rubella, chicken pox, flu) or a bacterial infection, in particular meningococcal, and also after vaccination and use of antibiotics, streptocides and nek-ry other pharmaceuticals. At the same time an extensive hemorrhagic enanthesis of extremities and a trunk with the subsequent ulceration and necroses of the most affected areas are noted. The necroses of skin over large joints which are quite often coming to an end with gangrene of an ulceration of a mucous membrane of a mouth are typical and profuse went. - kish. the bleedings which are usually leading to a lethal outcome. At adults the fulminant purpura is observed less than at children, and in each its case it is necessary to exclude a symptomatic vasculitis especially carefully.
The X ronichesky recurrent form Sh. — G. it is characterized skin, joint, abdominal and renal by syndromes. Various combinations of these syndromes are possible. The option of disease which is shown skin and joint syndromes (a skin and joint form), often call simple.
The skin syndrome is observed at most of adult patients while damage of kidneys, joints, went. - kish. a path less than in 50% of cases. At children a hemorrhagic enanthesis comes to light practically in all cases (96 — 98%) of a disease, the abdominal syndrome meets considerably more often than at adults (there are instructions that its frequency makes 54 — 72%); damage of joints is observed approximately in 42 — 59% of cases, fever — at 60 — 75% of the diseased, a renal syndrome — approximately in 24 — 66% of cases. A hemorrhagic enanthesis (purpura) can be located in the form of separate elements on nek-rum distance from each other or groups. Rashes arise wavy, and the first waves of rash happen the most rough, the subsequent are less expressed. Most often rash is localized symmetrically on a front surface of shins, feet, hips, buttocks, on an outer surface of shoulders and forearms, in a circle of the affected joints (elbow, knee, talocrural). Much less often the purpura arises on skin of a breast, a back, a front surface of a stomach, on a face, is extremely rare — on a mucous membrane of a mouth, a conjunctiva, centuries, auricles, lobes of ears. Rashes have papular and hemorrhagic character, is frequent about Ur-tikarnymi elements, during the pressing do not disappear. Their coloring changes depending on time of emergence. In the beginning rashes bright red, cyanotic-crimson, by the end of third day they, as a rule, pale yellow, and can disappear further absolutely. Quite often the purpura is followed by an itch that conducts to numerous raschesa; less often patients are disturbed by a burning sensation. Sometimes there are paresthesias, especially when rashes are localized on the back of feet and brushes. At elderly people of purple quite often has necrotic or violent and necrotic character. In this case disease heavy, long. Sometimes necrotic rashes on finger-tips of hands and legs come to an end with gangrene. Nek-ry patients (is more often at children and teenagers) can have Quincke's diseases (see Quincke swelled) on brushes, feet, lips, in eye-sockets, a pilar part of the head, the severe pain and unpleasant feelings causing sometimes.
In rare instances the vasculitis is localized in cartilaginous structures of auricles, a nose, joints, edges, and then the disease proceeds as a recurrent polychondritis with deformation of cartilages (see the Chondritis).
The joint syndrome often arises along with skin or several hours later or days after it. Pains are localized preferential in large joints — knee, talocrural, to-rye quite often swell. The joint syndrome in several days, as a rule, disappears, flexion contractures form seldom. Deformations and disturbances of mobility of joints, as a rule, do not come to light.
The abdominal syndrome dominates in a wedge, a picture approximately in V3 of cases, preceding sometimes emergence of skin rashes that complicates the correct diagnosis. Its main manifestation — the strong constants or colicy pains in a stomach caused by hypostasis and hemorrhages in a wall of a gut, a mesentery or a peritoneum. Pains arise suddenly, are more often localized around a navel, but also in right hypochondrium, the right ileal area, epigastriums can be defined, imitating acute impassability of intestines, perforation of an ulcer, appendicitis, cholecystitis, torsion of an oothecoma. Usually pains pass within 2 — 3 days, 7 — 10 days proceed less often, have character of attacks, to-rye repeat several times in days. At the same time patients are uneasy, rush about in a bed, often adopt the forced provision — lie, having writhed, on one side, having pressed the legs bent in knees to a stomach. Abdominal distention is often noted, but in some cases he can be involved, actively participates in the act of breath. At nek-ry patients the small tension of an abdominal wall comes to light. Quite often there is nausea, is more rare — a hematemesis. Approximately in half of cases fresh blood in Calais or a melena appears. At plentiful blood loss the collapse develops (see), acute posthemorrhagic ant iya (see). Frequent vomiting, ponosa can lead to dehydration, a hypoalbuminemia. At the height of a disease fever, a leukocytosis in this connection quite often resort to an operative measure concerning estimated acute patol are often noted. process in an abdominal cavity. Many clinical physicians consider that it is less dangerous, than to refuse in such situation operation as also the vasculitis can cause intestinal impassability (invagination), be complicated by perforation of an ulcer of a gut and peritonitis. The diagnostic laparotomy is carried out if trial treatment of a vasculitis does not give effect, and clinic of an acute abdomen we accrue.
The renal syndrome of various expressiveness proceeds as an acute or chronic glomerulonephritis (see), to-ry develops most often on 2 — the 4th week of a disease and is characterized by a microhematuria or a gross hematuria, a proteinuria, a cylindruria. Arterial hypertension is observed seldom. Risk of involvement in patol. process of kidneys is higher at men and teenagers. In several weeks or months from the moment of development the glomerulonephritis can disappear. Hron. the current of a glomerulonephritis substantially worsens the forecast in connection with a possibility of development of uraemia (see).
Duration of a current of a chronic form III. —. considerably varies and depends on expressiveness of defeat of this or that body.
The diagnosis establish on the basis of typical a wedge, signs and results of laboratory researches. In sick Sh.' blood — G. reveal (changeably) insignificant leukocytosis, acceleration of ROE, occasionally an eosinophilia. In
serum 400 ShENLEYNA - GENOH the DISEASE
usually raises contents the а2nd - and at - globulins, in plasma — the level of fibrinogen, quite often are found S-reactive protein, sialine to - you, often (approximately in 60% of cases) come to light increase in content in blood of cell-bound immune complexes.
The differential diagnosis is carried out with hard proceeding symptomatic vasculites at tuberculosis, malignant tumors (cancer, histiocytic sarcomas), with vasculites at a lymphogranulomatosis (see), Beck's sarcoidosis (see the Sarcoidosis), a pseudorheumatism (see), hron. active hepatitis (see), a long bacterial endocarditis (see the Endocarditis). The combination of a vasculitis to a frequent recurrence of a Quincke's disease demands an exception of a system lupus erythematosus (see), especially at persons is a female. At a fulminant purpura, and also at a long and heavy current of a vasculitis at adults first of all exclude limfoproliferativny diseases — a lymphoma, a lymphocytoma, a lymphosarcoma (see), a multiple myeloma (see), Valdenstrem's macroglobulinemia (see Valdenstrem a disease).
As the wedge, manifestations actually of a vasculitis have no etiological specificity, the differential diagnosis with symptomatic forms in each case is based on active identification of symptoms of an estimated disease.
Treatment. Obyazatelna hospitalization of patients and observance of a bed rest during the first 2 — 3 weeks of a disease. Exclude the sensibilizing foodstuff (a citrus, chocolate, fresh berries) from a diet. It is necessary to avoid use of medicines if the forecast of their effect is doubtful. Antibiotics are appointed only at the proved bacterial infection. Dimedrol, Suprastinum, tavegil and other antihistaminic drugs are not effective, as well as the drugs of calcium which were widely used earlier, ascorbic to - that, Rutinum. Use non-steroidal anti-inflammatory drugs (indometacin, Voltarenum). Glucocorticoid hormones, but to I. E. Tareeva's (1983) data, have in some cases favorable effect at skin and joint syndromes, but do not influence on patol. process in kidneys; at the same time they increase coagulability of blood, inhibit a fibrinolysis, aggravate an intravascular blood coagulation. Therefore they are appointed with care and only in a complex with heparin. Use of heparin is most effective in connection with multilateral effect of drug on pathogenetic factors of Sh. — G. He eliminates hypercoagulation and stops the disseminated blood coagulation, inhibits a complement and interferes with its further activation, stimulates a fibrinolysis. Therapy with heparin is begun after definition of a geparinorezistentnos-ta of in vitro plasma and out under control of indicators of coagulability of blood. Usually heparin is appointed at the rate of 300 — 400 PIECES to
1 kg of body weight a day in the form of injections Under skin of a stomach (each 6 — 8 hours), if necessary changing a dose so that to provide decrease in indicators of the autokoagulyatsionny test and the activated partial tromboplastinovy time twice. At development of geparino-resistance the dose of drug is increased (sometimes the daily dose is brought to 800 PIECES on 1 kg of body weight). If the geparinorezistentnost is connected with deficit of antithrombin III, then the patient daily enter into a vein struyno 300 — 400 ml of freshly frozen donor plasma. For improvement of microcirculation and strengthening of a fibrinolysis recommend intravenous drop administration nicotinic to - you in the most transferable dosages. As-zagreganta trental can be used.
The patient with an abdominal syndrome appoint full (!) hunger until pains are stopped. It is allowed to drink water only. At profuse bleeding from went. - kish. a path struyno pour high doses of freshly frozen plasma (to 1000 ml and more). Introduction aminocaproic to - you is contraindicated since it can lead to aggravation of a syndrome of the disseminated intravascular coagulation. It is inexpedient to appoint heparin and dezag-regant. In the absence of effect make a resection of the struck department of intestines.
To the most effective methods of treatment III. —. the plasma exchange belongs (see). The effect occurs quickly — after the first or second procedure. Even the most severe cryoglobulinemic forms of vasculites proceeding with a heavy syndrome of an intravascular blood coagulation are stopped. Hold usually 5 — 6 procedures in 3 — 4 days. For a session delete from 1500 to 2000 ml of plasma of the patient. Deficit of liquid and proteins fill salt r-ra-mi, reopoliglyukiny, 10% with solution of albumine, freshly frozen plasma. Both at acute, and at hron. forms Sh. — G. very effective was a so-called selection plasma exchange. The essence of a method consists in extracorporal sedimentation at t ° 4 ° macromolecular units, fibronectin, fibrinogen, the VIII blood-coagulation factors, krioglobuli-
ny, cell-bound immune complexes, fragments of cellular membranes are a part to-rykh. The listed and other components of plasma pre-tsipitirut in the presence of heparin. The plasma «purified» thus can be frozen and further is poured to the patient at the following gshazmaferez. At f — 20 ° the plastic container with plasma can be stored within a week. In the absence of effect of the listed above methods of treatment or at a frequent and heavy recurrence of III. —. appoint the cytostatic drugs possessing an immunodepressive effect: azatio-
prin on 150 mg a day within
3 — 4 months, Cyclophosphanum on 200 mg every other day (a total course dose of 8 — 10 g). Treatment by cytostatics needs to be carried out under control of level of leukocytes to blood. It is necessary to avoid use of these drugs at children, considering mutagen effect of tsitostatik.
The forecast depends on a form and severity, a disease. It is worse at a fulminant form, at surgical intervention at patients with an abdominal purpura, and also in case of development of uraemia at damage of kidneys.
Prevention of exacerbations of a disease consists in the prevention of infections, an exception of the use of the sensibilizing food stuffs and drugs, and also alcohol. To children after hospitalization rehabilitation in local sanatoria during 1V2 — 2 with the subsequent medical examination at the regional pediatrician is carried out of month. Strike off the dispensary register in 5 years after the last palindromia. According to medical indications release from inoculations for 2 years is given. Patients shall avoid overcooling and excess insolation.
Bibliography: B are kagan 3. S. Gemor
ragichesky diseases and syndromes, M., 1980; Vasilyev S.A., etc. Change of concentration of fibronectin in про^ to a tsessa of carrying out a medical plasma exchange, Rubbed. arkh., t. 56, No. 6, page 35, 1984;
Vorobyov A. I., Gorodetsky V. M. and Brilliant M. D. A plasma exchange in clinical practice, in the same place, page 3; Gorodetsky V. M. and P y to about V. V. Plazmaferez in therapy of the diseases caused by immune pathology, in the same place, page 19; Clinical nephrology, under the editorship of E. M. Tareeva, t. 2, page 184, M., 1983; Morphological bases of immunopathological processes and diseases, under the editorship of V. V. Serov, M., 1978;
Nasonova V. A. A hemorrhagic vasculitis (Shenleyn's Disease — Genokh), M., 1959, bibliogr.; it, the Pseudorheumatism in modern rheumatology, Rubbed. arkh., t. 50, No. 9, page 3, 1978; Savchenko V. G., etc. Level of plasma fibronectin at patients with diseases of system of blood, in the same place, t. 56, № 6, page 28, 1984; Modern hematology and oncology, under the editorship of J. Lobui, etc., the lane with English, page 105, M., 1983; I r y-gin N E., Nasonova V. A. and Funs on R. N. System allergic vasculites, M., 1980; Hematology, ed. by W. J. Williams a. o., p. 1393, N. Y., 1977; Henoch E.
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