CAJ | 학술논문

目的探讨原发性胆汁性肝硬化合并多发性肌炎的临床特点、鉴别诊断及其治疗.方法报道本院原发性胆汁性肝硬化、肝移植术后合并多发性肌炎一例并复习相关文献.结果患者为49岁女性,确诊原发性胆汁性肝硬化7年,肝移植术后1年出现肌无力、心悸,血清肌酶升高,肌电图、肌活检符合多发性肌炎表现.在排除其他免疫性疾病、药物性肌炎、肿瘤相关肌炎和移植物抗宿主反应后,临床诊断为多发性肌炎,加强免疫抑制治疗后病情好转.结论原发性胆汁性肝硬化肝移植后有可能合并多发性肌炎,但临床上应注意鉴别诊断,除外可能引起多发性肌炎的其他因素;治疗上应加强免疫抑制.
목적 탐토원발성담즙성간경화합병다발성기염적림상특점、감별진단급기치료.방법 보도본원원발성담즙성간경화、간이식술후합병다발성기염일례병복습상관문헌.결과 환자위49세녀성,학진원발성담즙성간경화7년,간이식술후1년출현기무력、심계,혈청기매승고,기전도、기활검부합다발성기염표현.재배제기타면역성질병、약물성기염、종류상관기염화이식물항숙주반응후,림상진단위다발성기염,가강면역억제치료후병정호전.결론 원발성담즙성간경화간이식후유가능합병다발성기염,단림상상응주의감별진단,제외가능인기다발성기염적기타인소;치료상응가강면역억제.
Objective To explore the clinical characteristics, differential diagnosis and treatment option of patients with polymyositis complicated with primary biliary cirrhosis. Method We report a case of polymyositis complicated with primary biliary cirrhosis after orthotopic liver transplantation with literature review. Results This is a 49-year-old female who had been diagnosed primary biliary cirrhosis for 7 years, developed myasthenia and palpitation 1 year after liver transplantation. The result of serum creatine kinase, electromyogram and musculi quadriceps femoris biopsy indicated polymyositis. She was given more tests to rule out other autoimmune diseases, neoplasm associated myositis, graft versus host disease and drug-induced myopathy. Finally she was diagnosed polymyositis and relieved by intensed immunosuppressant treatment. Conclusion Polymyositis may complicate with primary biliary cirrhosis, although it is extremely rare, in which ease ruling out other causes of myositis is important and intensed immunosuppressant is the right option.