Anti-neutrophil cytoplasmic antibody (ANCA)- associated vasculitides (AAV) are a group of debilitating autoimmune diseases characterized by uncontrolled inflammation and endothelial injury that often associates with pauci-immune necrotizing and crescentic glomerulonephritis. Despite the improved therapeutic options, both mortality and development of end-stage kidney disease remain high among AAV patients. 1 More importantly, complications associated with treatments continue to pose as major risk factors for infection, cardiovascular disease, and malignancy. Therefore, a better understanding of the disease patho- genesis is needed to develop more selective and safer, hypothesis-driven treatments.
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