[Necrotizing granulomatosis with pulmonary, intestinal and renal involvement].

CASE REPORT: 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.

CONCLUSION: 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.