From the Department of Rheumatology, and the Department of Surgery, Department of Clinical and Experimental Medicine, and the Department of Nephrology, Department of Medical and Health Sciences, Linköping University; Department of Rheumatology, and Department of Nephrology, County Council of Östergötland, Linköping, Sweden.
P. Eriksson, associate professor, Department of Rheumatology, Linköping University hospital; M. Segelmark, professor, Department of Nephrology, Linköping University hospital; O. Hallböök, professor, Department of Surgery, Linköping University hospital.
J Rheumatol. 2018 Apr;45(4):529-537. doi: 10.3899/jrheum.170249. Epub 2018 Feb 1.
Involvement of the gastrointestinal (GI) tract is a rare complication of granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). The aim was to describe frequency, diagnosis, treatment, and outcome of GI disease in a large series of patients in a single center.
A database that includes all patients with GPA and MPA diagnosed since 1997 in a defined area of southeastern Sweden as well as prevalent older cases and tertiary referral patients was screened for patients with GI disease. Data were retrieved from the patient's medical records, and GI manifestations of vasculitis were defined as proposed by Pagnoux, in 2005.
Fourteen (6.5%) of 216 consecutive patients with GPA/MPA had GI manifestations. Abdominal pain and GI bleeding were the most common symptoms. Radiology was important for detection of GI disease, while endoscopy failed to support the diagnosis in many patients. Because of perforation, 5 patients underwent hemicolectomy or small intestine resection. Primary anastomosis was created in 2/5 and enterostomy in 3/5 patients. One patient had a hemicolectomy because of lower GI bleeding. One sigmoid abscess was treated with drainage, and 1 intraabdominal bleeding condition with arterial coiling. Two patients died from GI disease. GPA and MPA patients with and without GI disease exhibited a similar overall survival.
GI disease was found in 6.5% among 216 patients with GPA or MPA. Surgery was judged necessary only in cases with GI perforation or severe bleeding. Multidisciplinary engagement is strongly recommended.
胃肠道（GI）受累是肉芽肿性多血管炎（GPA）和显微镜下多血管炎（MPA）的罕见并发症。本研究旨在描述单一中心内大量患者中 GI 疾病的发病频率、诊断、治疗和结局。
本研究筛选了自 1997 年以来在瑞典东南部一个特定区域内诊断的所有 GPA 和 MPA 患者以及已确诊的老年病例和三级转诊患者的数据库，以确定是否存在 GI 疾病患者。数据从患者的病历中提取，GI 血管炎表现根据 Pagnoux 于 2005 年提出的标准定义。
216 例连续 GPA/MPA 患者中有 14 例（6.5%）存在 GI 表现。腹痛和 GI 出血是最常见的症状。影像学对于发现 GI 疾病很重要，而内镜在许多患者中无法支持诊断。由于穿孔，5 例患者接受了结肠部分切除术或小肠切除术。2/5 例行一期吻合，3/5 例行肠造口术。1 例因下 GI 出血而行结肠部分切除术。1 例乙状结肠脓肿行引流术，1 例腹腔内出血行动脉栓塞术。2 例患者因 GI 疾病死亡。有或无 GI 疾病的 GPA 和 MPA 患者的总生存情况相似。
在 216 例 GPA 或 MPA 患者中，有 6.5%的患者存在 GI 疾病。只有在存在 GI 穿孔或严重出血的情况下，才需要进行手术。强烈建议多学科参与。
