Treatment of antineutrophil cytoplasmic antibody-associated vasculitis with rituximab.

PURPOSE OF REVIEW: To review the present knowledge about the use of rituximab (RTX) in patients with granulomatosis with polyangiitis (Wegener's; GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (Churg-Strauss; EGPA), also collectively referred to as antineutrophil cytoplasmic antibody-associated vasculitis.

RECENT FINDINGS: More than 20 case series and cohort studies involving more than 200 patients focusing on RTX use for patients with refractory GPA and MPA have been reported. Two randomized controlled trials have shown that RTX is not inferior to cyclophosphamide (CYC) for induction of remission in severe GPA and MPA. The RAVE trial has further shown that RTX is superior to CYC for patients with severe disease relapses. In addition, reports are emerging on the use of RTX for remission maintenance in chronically relapsing patients. There are also preliminary reports on the beneficial use of RTX in eosinophilic granulomatosis with polyangiitis (Churg-Strauss).

SUMMARY: RTX is the first proven alternative to CYC for remission induction in severe GPA and MPA. RTX is the preferred agent for patients presenting with severe disease flares, and its use had become the de facto standard of care for patients with chronically relapsing refractory GPA. Its use in EGPA requires further investigation.