Vasculitis comprises a heterogeneous group of diseases characterized by inflammation and destruction of blood vessels.
The aim of the study. This review highlights recent progress in epidemiology, etiology, pathogenesis of antineutrophil cytoplasmic antibody-associated (ANCA-associated) vasculitis: especially of Churg–Strauss syndrome, Wegener’s granulomatosis and microscopic polyangiitis.
Materials and methods. It was summarized information due to the distribution, trigger factors (genetic, infectious, toxic) and immunopathogenesis (immunocomplex, antineutrophil cytoplasmic antibody-associated, T- and B-lymphocyte-dependent, cytokines mechanisms) of ANCA-associated systemic vasculitis.
Results. It was found that the prevalence of ANCA-associated systemic vasculitis has a tendency to the growth in various Western European countries and has its differences over the world. The etiology of most ANCA-associated vasculitides is not yet clear. Their nomenclature and classification is therefore a challenge for internists, immunologists, and pathologists. In the same time it was found that infectious agents have been implicated in the etiopathogenesis of various ANCA-associated vasculitides via numerous and overlapping mechanisms including direct microbial invasion of endothelial cells, immune complex mediated vessel wall damage and stimulation of autoreactive B and/or T cells through molecular mimicry and superantigens.
Conclusion. As, it is mirrored by the availability of biological agents, which herald a revolution in the treatment of systemic vasculitis. As rare diseases, knowledge of which pathogenesis grows, treatment advances in ANCA-associated vasculitis depend on international collaborative research networks.