Wegener granulomatosis with massive intracerebral hemorrhage: remission of disease in response to rituximab.

Wegener granulomatosis (WG) is a multisystem necrotizing vasculitis that primarily involves the upper and lower respiratory tract and kidneys but can affect almost any organ, including the central nervous system (CNS). We present a patient with WG whose disease was complicated by a massive intracerebral hemorrhage (ICH) despite standard treatment with prednisone and cyclophosphomide. Although CNS involvement is not uncommon in WG, ICH is a rare complication of WG, and although the majority of patients with WG complicated by a cerebrovascular accident have a fatal outcome, our patient survived this event. The disease subsequently progressed with recurrent pulmonary involvement and renal failure. Our patient either did not tolerate or failed to respond to several immunosuppressive agents, including cyclophosphamide, methotrexate, and mycophenolate mofetil, but achieved remission after treatment with rituximab.