伴多发血管炎的肺肉芽肿性病（韦格纳肉芽肿病）与肠道克罗恩病并存
克罗恩病（CD）可能与不同的肠外表现有关，比如罕见的呼吸道上皮受累。当肺部出现渐进性坏死结节时，鉴别诊断包括多发血管炎性肉芽肿病（韦格纳肉芽肿病）（GPA），CD与GPA的呼吸道上皮病变表现非常相似，使得诊断变得困难并且提示这两种疾病共存的可能性，本研究旨在描述这两种疾病并存时的临床，放射学及病理学特征。我们回顾性研究了梅奥临床中心炎症性肠病和抗中性粒细胞胞浆抗体（ANCA）相关性血管炎共存患者的档案记录。排除了那些包括溃疡性结肠炎在内的只有一个诊断患者后，我们详细的回顾复习了97例患者的档案资料。对于那些可获得的肺和胃肠道活检进行了回顾性复习，我们也搜索以前报道的与这些病例有关的医学文献，发现了6例CD与肺GPA并存，4例与肺外GPA并存；3例文献报道的CD与肺外GPA并存。其中11例CD的诊断先于GPA的诊断。其中6例蛋白酶3-ANCA阳性，2例阴性，另外5例没有报道。6例髓过氧化物酶-ANCA阴性，其余7例未知。所有病例GPA的诊断，病理学都显示了特征性的渐进坏死性肉芽肿性炎和节段性血管炎。对于那些单存发现CD、肉芽肿性肠炎及GPA的患者中，要警惕CD和GPA并存的可能性。 Am J Surg Pathol.2014,38(3):354-359.

Abstract: Crohn disease (CD) may be associated with various extraintestinal manifestations, including, rarely, respiratory tract involvement. When necrobiotic pulmonary nodules are present, the differential diagnosis includes granulomatosis with polyangiitis (Wegener granulomatosis) (GPA). The respiratory tract manifestations of CD and GPA may mimic each other, complicating the diagnosis and suggesting the possible coex-istence of these 2 conditions. The aim of this study was to de-scribe the clinical, radiographic, and pathologic features of patients in whom CD and GPA coexist. We reviewed the teaching files of the authors and searched the Mayo Clinic medical records for coexistent inflammatory bowel diseases and antineutrophilic cytoplasmic antibodies (ANCA)-associated vasculitides. We reviewed in detail 97 patient charts and ex-cluded cases of ulcerative colitis and those in whom only one of the diagnoses was present. Pulmonary and gastrointestinal bi-opsies were reviewed when available. We also searched the medical literature for previously published cases. We found 6 cases of coexistent CD and pulmonary GPA and 4 cases with extrapulmonary GPA; 3 cases (all with extrapulmonary GPA) have been published previously. The diagnosis of CD preceded that of GPA in 11 cases. Proteinase 3-ANCA was positive in 6 cases, negative in 2, and not reported in 5 cases. Myeloperox-idase-ANCA was negative in 6 cases and unavailable in the re-mainder of patients. Pathology revealed features diagnostic of GPA in all cases with necrotizing granulomatous inflammation and segmental vasculitis. Pulmonary findings in patients with CD or the presence of granulomatous colitis in patients with GPA should prompt the inclusion in the differential diagnosis of a possible coexistence of CD and GPA.