Granulomatosis with polyangiitis and microscopic polyangiitis: pathogenesis and clinical manifestations
https://doi.org/10.14412/1996-7012-2025-4-86-95
Abstract
This literature review addresses the epidemiology, genetic markers, pathogenesis, clinical features, and diagnostic approaches of granulomatosis with polyangiitis and microscopic polyangiitis – both antineutrophil cytoplasmic antibody-associated vasculitides. Particular attention is paid to the role of neutrophil activation and the alternative complement pathway in disease development. The review also includes information on avacopan, an orally administered, selective C5a receptor (C5aR1) inhibitor that blocks the C5a complement component signaling pathway.
About the Authors
T. M. Reshetnyak
V.A. Nasonova Research Institute of Rheumatology;
Russian Medical Academy of Continuing Professional Education, Ministry of Health of the Russia
Russian Federation
Russian Federation
34A, Kashirskoye Shosse, Moscow 115522;
2/1, Barrikadnaya Street, Build. 1, Moscow 125993
E. N. Vorkel
V.A. Nasonova Research Institute of Rheumatology
Russian Federation
Russian Federation
34A, Kashirskoye Shosse, Moscow 115522
K. S. Nurbayeva
V.A. Nasonova Research Institute of Rheumatology
Russian Federation
Russian Federation
34A, Kashirskoye Shosse, Moscow 115522
A. M. Lila
V.A. Nasonova Research Institute of Rheumatology;
Russian Medical Academy of Continuing Professional Education, Ministry of Health of the Russia
Russian Federation
Russian Federation
34A, Kashirskoye Shosse, Moscow 115522;
2/1, Barrikadnaya Street, Build. 1, Moscow 125993
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Review
For citations:
Reshetnyak TM, Vorkel EN, Nurbayeva KS, Lila AM. Granulomatosis with polyangiitis and microscopic polyangiitis: pathogenesis and clinical manifestations. Sovremennaya Revmatologiya=Modern Rheumatology Journal. 2025;19(4):86-95. (In Russ.) https://doi.org/10.14412/1996-7012-2025-4-86-95
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