成人斯蒂尔病继发肝损害的临床分析

Clinical Analysis of Secondary Liver Damage in Adult-Onset Still's Disease

目的 分析成人斯蒂尔病继发肝损害的临床特点、治疗对策及预后转归.方法 收集2013年1月—2019年10月苏州大学附属第一医院57例成人斯蒂尔病住院患者,对患者的临床表现、肝损害情况、治疗及预后资料进行回顾性分析,并将肝酶水平与同期63例类风湿关节炎(RA)患者进行比较.结果 成人斯蒂尔病继发肝损害25例(43.9%),其中女性19例(76.0%),平均年龄(36.4±11.7)岁,平均病程(10.5±22.3)个月.与RA患者比较,肝损害发生率显著高于RA患者(43.9%vs 14.3%,P<0.05);天门冬氨酸氨基转移酶(AST)和碱性磷酸酶(ALP)水平显著高于RA患者(均P<0.05).其中,22例轻中度肝损害患者经过激素治疗后好转;3例重度肝损害患者,2例继发噬血细胞综合征,按照嗜血细胞综合征相关指南治疗后好转;另1例考虑噬血细胞综合征可能,转院后失访.结论 成人斯蒂尔病继发性肝损害常见,且明显高于RA患者;轻中度肝损害对糖皮质激素治疗敏感,但重度肝损害极易继发噬血细胞综合征,需及时诊断及调整治疗方案.

Objective To analyze the clinical features,treatment strategies and prognosis of adult-onset Still's disease(AOSD)with liver damage.Methods A total of 57 AOSD patients who were admitted to the First Affiliated Hospital of Soochow University,from January 2013 to October 2019 were enrolled.The clinical manifestations,treatment strategies and prognosis were retrospectively analyzed,and the liver enzymes were compared with patients with rheumatoid arthritis(RA)during the same period.Results 25(43.9%)of AOSD patients had liver damage,and in these patients,female patients were of 19(76.0%),average age of(36.4±11.7)years and average duration of disease(10.5±22.3)months.The incidence of liver damage in AOSD patients was significantly higher than that in RA patients(43.9%vs 14.3%)(P<0.05),and the levels of aspartate aminotransferase(AST)and alkaline phosphatase(ALP)in AOSD patients were significantly higher than that in RA patients(all P<0.05).22 patients had mild to moderate liver damage and significantly improved after glucocorticoids therapy.Among the 3 patients with severe liver damage,2 patients progressed to haemophagocytic lymphohistiocytosis(HLH)and improved after standardized treatment for HLH;and 1 patient had probable HLH but lost after the transfer.Conclusion The incidence of liver damage in AOSD patients is common and significantly higher than that in RA patients;patients with mild to moderate hepatic damage are responded well to glucocorticoid therapy,but patients with severe liver damage are prone to secondary HLH.It is necessary to diagnose and adjust the drug regimen in time for these patients.