中华临床免疫和变态反应杂志Issue(2)：108-112,5.DOI:10.3969/j.issn.1673-8705.2014.02.004
成人多发性肌炎或皮肌炎并发慢性假性肠梗阻的临床特点
Clinical Characters of Chronic Intestinal Pseudo-obstruction in Adults with Polymyositis/Dermatomyositis
摘要
Abstract
Objective To explore the clinical features of chronic intestinal pseudo-obstruction (CIPO)in adults with polymyositis (PM)or dermatomyositis (DM).Methods Six PM/DM patients with CIPO admitted to Peking Union Medical College Hospital(PUMCH)between November 2003 to October 201 3 were analyzed retrospectively.The clinical characteristics,the results of laboratory tests,treatment and prognosis of these patients were summarized.Results CIPO occurred in 0.45% (2/441 )of adult PM patients and 0.54%(4/747)of adult DMpatients respectively.The mean age of the 6 patients (two males and four females) was 41 ±1 5 years.CIPO was not the initial symptom.The onset of CIPO was from 1 year to 1 0 years after the diagnosis of PM/DM.Small bowel obstruction was identified by imaging examination in all patients.Five of six patients (83%)developed acute upper gastrointestinal bleeding.Multiple mucous ulcers located at lesser curvature of stomach and descending part of duodenum were observed under endoscopy in two patients.No muscular involvement was observed in 33% (2/6)of the patients at the onset of CIPO.Four of the 6 patients (67%) had normal creatine kinase,erythrocyte sedimentation rate,and high-sensitive C-reactive protein.Muscle biopsy of one patient suggested small blood vessel vasculitis.Five patients (83%)were given high-dose steroid (1 mg·kg -1 ·d -1 )and two of them were given steroid pulse therapy (1 g/d).Three of them (50%)finally recovered.Conclusions The pathogenesis of CIPO in adult PM/DM remains uncertain. It may be associated with secondary vasculitis. The acute complication of gastrointestinal bleeding leads to high morbidity and mortality and makes the decision making of treatment difficult. Early high-dose steroid and adjunctive immunosuppressive drugs may help to improve the prognosis.关键词
多发性肌炎/皮肌炎/慢性假性肠梗阻/消化道出血Key words
polymyositis/dermatomyositis/chronic-intestinal-pseudo-obstruction/gastrointestinal-bleeding分类
医药卫生引用本文复制引用
焦洋,张文,谷俊杰,费允云,徐娜,曾学军..成人多发性肌炎或皮肌炎并发慢性假性肠梗阻的临床特点[J].中华临床免疫和变态反应杂志,2014,(2):108-112,5.基金项目
北京市科技新星 （）
