Granulomatosis with polyangiitis with intestinal involvement successfully treated with rituximab and surgery

Intestinal perforation is a rare and life-threatening complication in granulomatosis with polyangiitis (GPA). A 55-year-old Japanese man who had been undergoing maintenance haemodialysis was diagnosed with GPA via a pathologically proven nasal granuloma and proteinase 3-antineutrophil cytoplasmic antibody-positive status. He was started on 60 mg prednisolone. Haematochezia was observed due to the colon ulcer after treatment initiation. Two doses of intravenous cyclophosphamide were administered every 2 weeks. Despite the treatment, there were two episodes of severe abdominal pain with peritonitis within a week. Perforation of descending colon and severe ischaemia of the ascending colon was observed during each emergency laparotomy, and ileostomy was performed. Vasculitis in the small vessels was confirmed. In conclusion, patient with severe intestinal involvement and who was unresponsive to conventional therapy was treated with surgery followed by rituximab administration, and remission was achieved.