Multiple ileal perforations in a patient with Wegener's granulomatosis: a case report and literature review.

INTRODUCTION: Wegener's granulomatosis (WG) is a chronic, multisystemic disease of unknown etiology characterized by necrotizing vasculitis and granulomatous inflammation. WG primarily involves the upper and lower respiratory tract and kidneys, but it may also affect multiple other organs or tissues, including the gastrointestinal system. DISCUSSION: Gastrointestinal involvement is an extremely rare manifestation of this disease. Moreover, during the course of WG, intestinal perforation is extremely rare in patients with gastrointestinal involvement. To our knowledge, only 13 WG cases with intestinal perforation have been reported in the English language literature as of September 2011. CASE REPORT AND LITERATURE REVIEW: We herein present the case of a 47-year-old male patient with WG who was diagnosed with multiple ileal perforations and ileovesical fistulae. The exact pathogenesis of intestinal perforation in WG is not fully understood. However, early surgical intervention and appropriate management with immunosuppressive therapy can be important to lifesaving measures. A review of 13 cases reported in the English language literature is also discussed, together with the pathogenesis of this serious complication.