特发性炎性肌病误诊为肝损伤25例临床分析  

Clinical Analysis of Misdiagnosis of 25 Cases of Idiopathic Inflammatory Myopathies as Liver Disease

作　　者：姚冬云[1] 霍河水[1] 赵文敏[1] 秦晨曼 魏秋瑾[1] 孙凯[1] 

机构地区：[1]焦作市人民医院风湿免疫科,河南焦作454002

出　　处：《临床误诊误治》2015年第6期14-17,共4页Clinical Misdiagnosis & Mistherapy

摘　　要：目的：探讨特发性炎性肌病（ idiopathic inflammatory myopathies, IIM）误诊为肝损伤的原因,以提高IIM诊治水平。方法回顾分析曾长期误诊为肝损伤的IIM 25例的临床表现、肌酶谱、炎性指标及随访情况。结果本组表现为发热19例（76%）,有典型向阳疹16例（64%）,肌痛18例（72%）；对称性四肢近端肌无力24例（96%）。肌电图异常24例。肌活检异常15例。胸部HRCT证实伴不同程度的间质性肺疾病19例（76%）。25例血清肌酸激酶（CK）、天冬氨酸转氨酶（AST）、丙氨酸转氨酶（ALT）均升高,AST/ALT均〉1,AST与ALT升高与CK增高呈显著正相关（r=0．934、0．865,P〈0．01）。误诊为药物性肝损伤3例,不明原因肝损伤22例；误诊时间3~11个月。均结合临床表现及自身抗体、肌电图、胸部 HRCT 检查确诊并予糖皮质激素联合免疫抑制剂治疗。随访0．5~5年,20例（80%）病情平稳,2例（8%）糖皮质激素减量后复发,3例（12%）因间质性肺疾病伴感染致呼吸衰竭死亡。结论 IIM患者临床表现特殊,AST与ALT升高与CK增高呈正相关。对IIM认识不足是本组误诊的主要原因,提高基层医院医师对该病的认识水平是避免误诊的关键。Objective To investigate the cause of misdiagnosis of idiopathic inflammatory myopathies（ IIM） as liver disease to improve diagnosis and treatment. Methods The clinical symptom, serum muscle enzymes, inflammatory parame-ters, and follow-up information of 25 cases confirmed as IIM which had long been misdiagnosed as hepatic injury were retro-spectively studied. Results There were 19（76%） patients who ran a fever in this study. Characteristic rash,muscle pain, symmetric proximal muscle weakness,diffuse interstitial lung disease（ILD） were present in 64%（16/25）, 72%（18/25）, 96%（24/25）, 76%（19/25）of all the patients. Electromyography（EMG） showed myogenic abnormality in 96% （24/25）of the patients. Muscle biopsy showed myogenic abnormality in 60%（15/25）of the patients. Serum muscle enzymes showed ris-ing concentration of all the cases （ 25/25 ） cases of creatine kinase （ CK ） , aspartate aminotransferase （ AST ） , alanine amin-otransferase（ALT）. The ratios of AST to ALT of all the cases were greater than 1. There was a significant liner correlation be-tween AST and CK（r=0. 934,P〈0. 01）, or between ALT and CK（r=0. 865, P〈0. 01）;3 cases were misdiagnosed as drug-induced liver injury, 22 cases as unexplained liver injury. 25 cases were confirmed by clinical manifestations, autoanti-bodies, EMG, chest HRCT examination and were treated with corticosteroids and immunosuppressive agents after misdiagnosis for 3~11 months. During （0. 5~5）-year-follow-up, 20（80%） patients achieved persistent remission, 2（8%） patients had a relapse when corticosteroids were tapered,3 patients（12%） died of ILD with infection. Conclusion With specific clinical manifestations in IIM, AST and ALT elevation was positively correlated with increased CK. The main cause of misdiagnosis is due to lack of awareness of IIM. Improved awareness of the disease by clinicians in local hospitals will help to prevent misdiag-nosis of IIM.

关 键 词：特发性炎性肌病 误诊 肝损伤 肌酸激酶 

分 类 号：R593.2[医药卫生—内科学]