[Acute abdomen in a patient with ANCA-associated vasculitis]

A 49-year-old man complained of increasing pain in the lower left abdomen. Three weeks previously joint pain had developed, and in the last 7 days the patient had noted a cutaneous rash at the lower legs. Within three days after admission a paralytic ileus developed, progressed and culminated in a small bowel perforation. In the 60 cm ileum specimen as well as in the skin lesions there was marked intra- und perivascular infiltration with neutrophil granulocytes and focal necrosis, but no granuloma.

As the proteinase 3 subtype of antineutrophil cytoplasmic antibodies (ANCA) was positive ANCA-associated vasculitis with gastrointestinal, cutaneous and kidney involvement was diagnosed. After initiation of cytostatic treatment with methylprednisolone boli und cyclophosphamide the patient's condition improved. The post-operative course was uneventful.

ANCA-associated vasculitis rarely presents with severe gastrointestinal complications. The disease represents an interdisciplinary challenge because of its variable clinical presentation and the possibly lethal outcome if not adequately treated.