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

摘要

目的：探讨成人多发性肌炎/皮肌炎（polymyositis/dermatomyositis，PM/DM）合并慢性假性肠梗阻（chronic intestinal pseudo-obstruction，CIPO）患者的临床特点及预后，以提高对此类疾病的认识。方法收集北京协和医院2003年11月至2013年10月临床成人 PM/DM合并 CIPO 患者的临床资料，回顾性分析及总结临床表现、实验室检查、诊治及转归。结果合并 CIPO 的成人 PM患者2例，DM患者4例，分别占同期住院成人 PM/DM患者的0．45％（2/441）及0．54％（4/747）。6例患者男女比例为1∶2，平均年龄（41±15）岁。CIPO 均非临床首发症状，发病时间为 PM/DM诊断后1～10年，部位均位于小肠。83％（5/6）患者病程中合并出现急性上消化道出血，2例患者经胃镜明确出血部位分别为胃小弯及十二指肠降部，镜下均表现为多发黏膜溃疡出血。6例患者中33％（2/6）在出现肠梗阻时并无明确肌肉受累表现，67％（4/6）肌酸激酶、红细胞沉降率及超敏 C 反应蛋白均处于正常范围。1例患者发病时肌肉活检示小血管炎性病理改变。6例患者中5例接受足量激素（1 mg/kg）治疗，2例接受大剂量激素冲击（甲泼尼龙1 g/d）联合静脉用人免疫球蛋白治疗，50％（3/6）患者最终完全缓解。结论成人 PM/DM患者合并 CIPO 发病机制尚不清楚，不除外与继发血管炎有关，由于病程中通常合并急性消化道出血，临床处理非常困难，总体死亡率高。早期识别并及时给予大剂量糖皮质激素和免疫抑制剂可能有助于改善预后。%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.