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The rise of complement in ANCA‐associated vasculitis: from marginal player to target of modern therapy
Author(s) -
Trivioli G.,
Vaglio A.
Publication year - 2020
Publication title -
clinical & experimental immunology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.329
H-Index - 135
eISSN - 1365-2249
pISSN - 0009-9104
DOI - 10.1111/cei.13515
Subject(s) - complement system , immunology , c5a receptor , vasculitis , anaphylatoxin , alternative complement pathway , medicine , classical complement pathway , complement (music) , glomerulonephritis , disease , antibody , biology , pathology , biochemistry , complementation , kidney , gene , phenotype
The complement system is critical for the pathogenesis of ANCA‐associated vasculitis (AAV). Complement activation via the alternative pathway is triggered by ANCA‐induced neutrophil degranulation and leads to the generation of C5a, a potent anaphylatoxin that enhances recruitment and priming of further neutrophils. Avacopan, an oral inhibitor of the C5a receptor, disrupts this pathogenic loop and may allow glucocorticoid‐free disease control, marking a new era in the treatment of AAV.

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