CAJ | 학술논문

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。
목적：탐토특발성염성기병（ idiopathic inflammatory myopathies, IIM)오진위간손상적원인,이제고IIM진치수평。방법회고분석증장기오진위간손상적IIM 25례적림상표현、기매보、염성지표급수방정황。결과본조표현위발열19례（76%),유전형향양진16례（64%),기통18례（72%)；대칭성사지근단기무력24례（96%)。기전도이상24례。기활검이상15례。흉부HRCT증실반불동정도적간질성폐질병19례（76%)。25례혈청기산격매（CK)、천동안산전안매（AST)、병안산전안매（ALT)균승고,AST/ALT균>1,AST여ALT승고여CK증고정현저정상관（r=0．934、0．865,P<0．01)。오진위약물성간손상3례,불명원인간손상22례；오진시간3~11개월。균결합림상표현급자신항체、기전도、흉부 HRCT 검사학진병여당피질격소연합면역억제제치료。수방0．5~5년,20례（80%)병정평은,2례（8%)당피질격소감량후복발,3례（12%)인간질성폐질병반감염치호흡쇠갈사망。결론 IIM환자림상표현특수,AST여ALT승고여CK증고정정상관。대IIM인식불족시본조오진적주요원인,제고기층의원의사대해병적인식수평시피면오진적관건。
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.