[Necrotizing granulomatosis with pulmonary, intestinal and renal involvement]

In a 45 year old patient pulmonary, renal and ocular manifestations of ANCA-associated vasculitis is reported that required immunosuppressive therapy. On admission the patient complained about enduring lower abdominal pain. A CT scan revealed free intraabdominal fluid and dilated small intestine filled with fluid. Laparotomy was performed with the working diagnosis of paralytic ileus. Intraoperatively, hemorrhagic-necrotic alterations of the small intestinal wall were conspicuous and resected. Microscopic examination revealed transmural ischemic necrosis of the resected intestinal tissue with prominent granulomatous vasculitis of arteries. CD20-antibody rituximab was applied due to the life-threatening condition and as ultima ratio therapy. Subsequently the disease activity was controlled, renal function improved and abdominal discomfort subsided.

Gastrointestinal involvement with necrotizing vasculitis is an uncommon but serious complication. Most patients respond to established therapy protocols encompassing cyclophosphamide and glucocorticoids. Administration of rituximab may be a promising alternative in refractory cases.