Treatment of granulomatosis with polyangiitis (Wegener's)

Granulomatosis with polyangiitis is a systemic necrotizing vasculitis characterized by granulomatous inflammation of small vessels and is associated with autoantibodies to neutrophil cytoplasmic proteases, mainly proteinase 3. Potentially lethal if not promptly diagnosed and treated, most patients with granulomatosis with polyangiitis can achieve remission with the current treatment modalities, with fewer side effects compared to three decades ago. However, the risk of relapse remains high, necessitating prolonged maintenance immunosuppressive therapy whose optimal duration remains undetermined. We review herein the treatment modalities for granulomatosis with polyangiitis and how they have evolved over the past decades. The findings of the most important and recently completed therapeutic studies, including on rituximab for maintenance, are summarized, before describing the main ongoing studies aimed at further optimizing treatment strategies.