Treatment of ANCA-associated vasculitis

Treatment of antineutrophil cytoplasmic autoantibody-associated vasculitides is tailored according to disease stage and severityCurrent treatments for granulomatosis with polyangiitis and microscopic polyangiitis do not reflect the fact they are genetically distinct diseasesFor remission induction, dose reduction and avoidance of prolonged use of cyclophosphamide have been successfully implemented; the addition of plasma exchange has increased the rate of renal recovery in patients with rapidly progressive glomerulonephritisMaintenance of remission treatment to prevent relapse is even more important when reduced amounts of cyclophosphamide are used during induction of remissionRituximab is noninferior to cyclophosphamide for remission induction, but its use as maintenance therapy is currently under investigation in randomized trialsIn patients with life-threatening disease, severe renal involvement and/or alveolar haemorrhage, plasma exchange can be successfully used as adjunctive therapy