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What provokes systemic vasculitis?
Last reviewed: 04.07.2025
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Causes of systemic vasculitis
Systemic vasculitis develops in children with altered reactivity. Among the factors contributing to its occurrence, the most significant are: frequent acute infectious diseases, foci of chronic infection, drug allergy, hereditary predisposition to vascular or rheumatic diseases. Bacterial or viral infections (streptococcal, hepatitis B or C, viral, herpesvirus, parvovirus), allergic reactions as a background for sensitization, a factor that allows or maintains activity are discussed by specialists with an emphasis on both infection and allergy.
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Pathogenesis of systemic vasculitis
In the pathogenesis, immune complexes circulating in the blood and deposited on the walls of blood vessels, immune inflammation with proliferative-destructive changes in the walls of arteries of various calibers, damage to the vascular endothelium, increased vascular permeability, hypercoagulation, and ischemia in the area of vascular damage are of great importance. Recently, in the pathogenesis of a number of systemic vasculitides, great importance has been attached to the fact of the formation of angina-neutrophil cytoplasmic antibodies (ANCA). These are organ-specific antibodies that react with various components of the cytoplasm of neutrophils and monocytes. In the presence of cytokines, ANCA cause degranulation of neutrophils, adhesion and damage to vascular endothelial cells, and stimulate the proliferation of T-lymphocytes, contributing to granulomatous inflammation. There are 2 types of antibodies:
- cANCA and pANCA. cANCA are antibodies that cause diffuse (classical) cytoplasmic luminescence. pANCA are antibodies that cause perinuclear luminescence of the neutrophil cytoplasm, more specific in relation to myeloperoxidase and other neutrophil enzymes. cANCA is detected in the active period in 90% of patients with Wegener's granulomatosis;
- pANCA - in 60% of patients with microscopic polyangiitis, 50% with Churg-Strauss syndrome, 15% with Wegener's granulomatosis. Therefore, in working classifications of systemic vasculitis, microscopic polyarteritis, Wegener's granulomatosis, and Churg-Strauss syndrome are currently classified as ANCA-associated vasculitis.