Complement system

Antibody

Vasculitis

Inflammation

Alternative complement pathway

Disease

Pathology

Immunology

Anti-neutrophil cytoplasmic antibody

Medicine

Anaphylatoxin

Nature Reviews Nephrology

Accumulating evidence indicates that activation of the complement system is crucial for the development of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). This Review provides an overview of the role of complement activation in AAV, and discusses how targeting this pathway can provide opportunities for treatment. Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of potentially life-threatening autoimmune diseases. The main histological feature in the kidneys of patients with AAV is pauci-immune necrotizing crescentic glomerulonephritis with little immunoglobulin and complement deposition in the glomerular capillary walls. The complement system was not, therefore, initially thought to be associated with the development of AAV. Accumulating evidence from animal models and clinical observations indicate, however, that activation of the complement system — and the alternative pathway in particular — is crucial for the development of AAV, and that the compl...

Complement in ANCA-associated vasculitis: mechanisms and implications for management