Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a group of rare systemic diseases affecting small-caliber vessels. The damage caused by AAV mainly involves the lung and kidneys. AAV includes three different types: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). Although the different phenotypic forms of AAV share common features, recent studies have shown that there are significant differences in terms of pathogenetic mechanisms involving both the adaptive and innate immune systems. Advances in our understanding of pathogenesis have enabled the development of immuno-targeted therapies. This review illustrates the characteristics of the various forms of AAV and the new therapies available for this disease that can have lethal consequences if left untreated.

New insights into pathogenesis and treatment of anca-associated vasculitis. autoantibodies and beyond / Paroli, Marino; Gioia, Chiara; Accapezzato, Daniele. - In: ANTIBODIES. - ISSN 2073-4468. - 12:1(2023). [10.3390/antib12010025]

New insights into pathogenesis and treatment of anca-associated vasculitis. autoantibodies and beyond

Marino Paroli
;
Chiara Gioia;daniele Accapezzato
2023

Abstract

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a group of rare systemic diseases affecting small-caliber vessels. The damage caused by AAV mainly involves the lung and kidneys. AAV includes three different types: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). Although the different phenotypic forms of AAV share common features, recent studies have shown that there are significant differences in terms of pathogenetic mechanisms involving both the adaptive and innate immune systems. Advances in our understanding of pathogenesis have enabled the development of immuno-targeted therapies. This review illustrates the characteristics of the various forms of AAV and the new therapies available for this disease that can have lethal consequences if left untreated.
2023
B-cells: complement; eosinophils; neutrophils; pathogenesis; vasculitis
01 Pubblicazione su rivista::01g Articolo di rassegna (Review)
New insights into pathogenesis and treatment of anca-associated vasculitis. autoantibodies and beyond / Paroli, Marino; Gioia, Chiara; Accapezzato, Daniele. - In: ANTIBODIES. - ISSN 2073-4468. - 12:1(2023). [10.3390/antib12010025]
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/1677505
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