Abstract
The review presents the recent data on assumed risk factors for the development of ANCA-associated vasculitis (AAV), among which environmental factors, such as climatic, chemical, etc., are of particular interest of researchers. Controversial opinions of various authors on the role of individual causative agents of infectious diseases in the development of AAV are analyzed. The review pays special attention to scientific data on the influence of variants of the structure of genes encoding various components of the immune system on the development of the pathogenetic process of AAV. Up-to-date information on the association of single-nucleotide polymorphisms (SNPs) with the course, risk of development and the likelihood of AAV recurrence is indicated, the most associated of which are genes encoding proteins of the main histocompatibility complex (HLA), a toll-like receptors (TLR`s), as well as an inhibitor of serine proteinases-alpha-antitrypsin (AAT). The analysis of scientific publications describing the molecular mechanism of the development of a pathological focus that forms the conditions for the synthesis of PR3ANCA and MPOANCA complexes characteristic of AAV has been carried out. The data of a number of foreign studies on the relationship of individual SNPs associated with the features of the course of granulomatosis with polyangiitis, microscopic polyangiitis, as well as eosinophilic granulomatosis with polyangiitis are presented and summarized. The review presents current AAV treatment regimens and promising directions for the development of medical care for patients.
Publisher
Paediatrician Publishers LLC