成人斯蒂尔病伴严重肝功能异常临床分析7例

Analysis of seven cases of adult onset Still's disease with severe liver injury

目的:总结成人斯蒂尔病(adult onset Still's disease,AOSD)伴严重肝功能异常患者临床特征,提高对该病的认识.方法:回顾性分析北京佑安医院2000年以来诊断为AOSD、同时伴有严重肝功能异常的患者临床资料,总结患者发生严重肝功能异常的可能原因.结果:7例患者病程10 d-5年,全部出现发热(100%);伴随或相继出现的症状包括:皮疹(100%)、咽痛(71.4%)、关节痛(71.4%)、淋巴结肿大(42.8%)、肝大和/或脾大(71.4%),严重肝功能异常(100%),合并亚急性肝衰竭1例,合并噬血综合征1例.AOSD伴严重肝功能异常可能与糖皮质激素剂量或疗程不够、感染和/或药物诱发、未能及时正确诊断、延误治疗有关.结论:加强对AOSD的认识,早期诊断、重视治疗过程的管理有助于减少重症肝损伤的发生.

AIM： To analyze the clinical characteristics of seven cases of adult onset Still＇s disease（AOSD） with severe liver injury to raise the awareness of the disease.METHODS： The clinical data for seven patients with AOSD with severe liver injury were obtained since 2000 and retrospectively analyzed to find the possible reason of severe liver injury in these patients.RESULTS： The duration of disease in the seven patients lasted from 10 d to 5 years. The patients presented with fever（100%）, rash（100%）, sore throat（71.4%）, arthralgia（71.4%）, lymphadenopathy（42.8%）, hepatomegaly and/or splenomegaly（71.4%）, or severe liver dysfunction（100%）. One case progressed into acute liver failure, and another case presented with hemophagocytic syndrome. AOSD with severe liver dysfunction might be related to insufficient dosage of glucocorticoid or inadequate treatment, infection and/or drugs, and delayed diagnosis or treatment.CONCLUSION： Raising the awareness of AOSD with liver injury, early diagnosis, and timely treatment and management might help to reduce the incidence of severe liver injury.