Severe gastrointestinal inflammation in adult dermatomyositis: Characterization of a novel clinical association
Severe gastrointestinal inflammation in adult dermatomyositis: Characterization of a novel clinical association
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DOI：
10.1097/00000441-200612000-00003
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发表时间：
2006-12-01
影响因子：
3.1
通讯作者：
Langevitz, Pnina
中科院分区：
文献类型：
--
作者：
Tweezer-Zaks, Nurit;Ben-Horin, Shomron;Langevitz, Pnina
目的：成人皮肌炎(DM)和多发性肌炎(PM)的胃肠道损害通常是轻微的，由肌肠动力障碍引起。成人DM和PM患者的严重消化道炎症很少见。本研究旨在探讨糖尿病患者炎性胃肠道损害的患病率和临床特点。方法：对1984-2004年间我科风湿科门诊收治的多发性肌炎或皮肌炎病例的病历进行回顾性分析，以明确严重的胃肠道损害。记录所有患者的临床病程和组织病理学结果，并计算这种疾病的患病率。结果：在48例DM或PM患者中，3例合并有严重胃肠道症状(占研究人群的6%)。胃镜下主要表现为肠壁水肿性充血、多发性糜烂和溃烂，组织学表现为弥漫性黏膜炎症和多发性血管扩张，无血管炎。由此产生的临床过程以反复腹痛和血性腹泻为显著特征，尽管接受了积极的免疫抑制治疗，但两名患有致命胃肠道穿孔的患者最终灾难性地结束了。结论：严重炎症性胃肠道疾病应被认为是成人糖尿病的一种严重的、尽管罕见的表现，预示着不良的预后和高致命性并发症的发生率。血管病变在该综合征发病机制中的作用仍有待确定。
Object: Gastrointestinal involvement in adult dermatomyositis (DM) and polymyositis (PM) is usually mild, resulting from myoenteric dismotility. Severe inflammation of the alimentary tract in cases of adult DM and PM is rare. The purpose of this study was to examine the prevalence and clinical characteristics of inflammatory gastrointestinal involvement in patients with DM. Methods: The charts of all cases with polymyositis or dermatomyositis, registered in our rheumatology clinic between 1984 and 2004, were reviewed retrospectively for documentation of severe gastrointestinal involvement. The clinical course and the histopathologic findings in all the patients were noted, and the prevalence of this disorder was computed. Results: Among 48 patients with DM or PM, 3 patients with DM and severe gastrointestinal tract manifestations were identified (6% of the study population). Edematous hyperemic bowel wall, with multiple erosions and ulcerous lesions were the prominent endoscopic findings, whereas diffuse mucosal inflammation and multiple vascular ectasias without vasculitis dominated the histologic picture. The resulting clinical course was notable for recurrent abdominal pain and bloody diarrhea, ending catastrophically in two patients with fatal gastrointestinal perforations, despite aggressive immunosuppressive therapy. Conclusions: Severe inflammatory gastrointestinal tract disease should be recognized as a grave, albeit rare, manifestation of adult DM that portends a poor prognosis and carries a high rate of fatal complications. The role of vasculopathy in the pathogenesis of this syndrome remains to be determined.