Rituximab for rescue and maintenance therapy in rapidly progressive life-threatening antineutrophil cytoplasmic autoantibody-associated systemic vasculitis.

Antineutrophil cytoplasmic autoantibody (ANCA)-associated systemic vasculitides (AASV) consists of small-vessel systemic inflammatory disorders which commonly affect the kidneys and without treatment have a poor prognosis. Rituximab is a novel biological agent which is being used experimentally in the management of AASV. This report presents the case of a young woman with rapidly progressive life-threatening AASV. Despite prompt diagnosis and initial treatment with steroids and alkylating agents her condition became life threatening. With addition of rituximab therapy she showed an excellent sustained response. Rituximab appears an effective and safe treatment choice for the induction of remission in severe AASV that is not responding to standard agents, at the initial presentation and for maintenance therapy, without the development of common serious side-effects associated with immunosuppression.