HEMORRHAGIC SCHÖNLEIN — HENOCH VASCULITIS, OCCURRING IN CHILDHOOD AND ADULTHOOD
Summary. Background. The typical beginning of hemorrhagic vasculitis (HV) Schönlein — Henoch in childhood included in the diagnostic criteria of the disease, although its further evolution and characteristics of pathological process with the debut of the disease in adult patients remains unclear. Objective. To assess the evolution of clinical, instrumental and morphological signs of lesions of the skin, joints and kidneys in patients with juvenile HV and recurrent course of the disease that began in childhood and adulthood, to study the nature of the disease pathogenic constructions in these groups of patients. Material and methods. The study included 174 patients with HV (83% men and 47% women). In 92 patients, the disease made its debut in childhood (on average in 12 years), and in 82 — in the adult (on average in 25 years). I, II and III degree of activity of pathological process are set at a ratio of 1:2:2. Results. In the evolution of juvenile HV cutaneous, cutaneous-joint-abdominal and cutaneous-abdominally-renal forms of the disease, affection of wrist, ankle and knee joints become more rarely, but renal variant of the pathological process become more often, developed chronic kidney disease, occur sacroiliitis, spondylopathy, tendovaginitis, enteropathies and epiphyseal osteoporosis. Urticarial form of skin syndrome peculiar to diseases which transformed from juvenile HV in adults and for this type of HV is unusual necrotic-ulcerative form with infiltration of skin by polymorphonuclear leukocytes and development of vasotrombosis. Adult patients are undergone by arthropathy more severely with the additional clinical, radiographic and sonographic signs, IV and V morphological classes of Henoch glomerulonephritis with tubulointerstitial component are formed in all cases. The age dimorphism in the onset of HV reveals lower serum levels of fibronectin, circulating immune complexes, rheumatoid factor, and endothelin-1, but higher concentrations of thromboxane A2, cyclic guanosine monophosphate and parameter of the surface viscosity, the incidence of eosinophilic infiltration and fibrinous necrosis of the skin vessels, extravasation of red blood cells and the deposition of fibrinogen. Conclusions. In cases transfer from juvenile HV to chronic adult form the disease often acquire progressive course that primarily relates to joints and kidney pathology.
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