Department of Surgery, Diyarbakir Education and Research Hospital, 21400, Diyarbakir, Turkey.
J Gastrointest Surg. 2012 Apr;16(4):857-62. doi: 10.1007/s11605-011-1735-z. Epub 2011 Nov 1.
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.
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.
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.
韦格纳氏肉芽肿病（WG）是一种病因不明的慢性、全身性疾病，其特征为坏死性血管炎和肉芽肿性炎症。WG 主要累及上、下呼吸道和肾脏，但也可能影响多个其他器官或组织，包括胃肠道。
胃肠道受累是这种疾病的一种极其罕见的表现。此外，在 WG 病程中，胃肠道受累的患者发生肠穿孔极为罕见。据我们所知，截至 2011 年 9 月，英文文献中仅报道了 13 例 WG 合并肠穿孔的病例。
我们在此报告一例 47 岁男性 WG 患者，该患者被诊断为多发性回肠穿孔和回肠膀胱瘘。WG 中肠穿孔的确切发病机制尚未完全阐明。然而，早期手术干预和适当的免疫抑制治疗管理对于挽救生命至关重要。我们还对英文文献中报道的 13 例病例进行了回顾，并讨论了这种严重并发症的发病机制。
