Eosinophilic granulomatosis with polyangiitis with severe pulmonary hemorrhage treated with rituximab.

Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by systemic vasculitis, asthma and eosinophilia. Severe pulmonary hemorrhage is rare. Renal involvement is seen in approximately 25% and can vary from isolated urinary abnormality to rapidly progressive glomerulonephritis. There is limited evidence to support the use of rituximab in this condition. We present a patient with EGPA who had severe pulmonary hemorrhage and rapidly progressive glomerulonephritis. He responded to standard treatment including prednisolone, cyclophosphamide, and plasma exchange. He subsequently had a relapse of pulmonary hemorrhage that was treated successfully with rituximab.