成人Still病与急性重度肝损害

Adult-onset Still's disease and acute severe hepatic lesion

目的探讨成人Still病并发急性重度肝损害的发生、发展,提高临床医生对成人Still病急性重度肝损害的认识。方法收集1990-2006年北京中日友好医院风湿免疫科诊治的104例(102例住院和2例门诊患者)成人Still病的患者资料,并对其临床表现、实验室检查,特别是10例并发急性重度肝损害的发生及发展情况进行了回顾性分析。结果102例住院诊治的成人Still病有54例(52.9%)出现不同程度肝损害,其中8例(7.8%)在治疗过程中出现急性重度肝损害,加上2例门诊随诊的重度肝损害患者,丙氨酸转氨酶均≥1000 U/L,总胆红素40～250 mg/L。10例患者在患病初期均有轻度肝损害,因不同原因加用常规剂量抗生素或免疫抑制剂或非甾体抗炎药,而出现急性重度肝损害。3例重度肝损害发展到肝功能衰竭而死亡,7例经激素加量或大量激素、保肝等治疗,肝功能恢复正常。结论成人Still病伴发轻度肝损害较常见,治疗过程中可因机体对某种药物的过度免疫反应,发生急性重度肝损害,因此对伴有轻度肝损害者的Still病患者用药一定要慎重。发生重度肝损害积极激素加量治疗,可以改善预后。

Objective To investigate the occurrence and progress of acute severe hepatic lesion in adult onset Still＇s disease （AOSD）, and understand the acute severe hepatic lesion in AOSD. Methods Dada of 104 AOSD patients＇ （102 cases were inpatient and 2 cases were outpatient） from 1990-2006 in China- Japan friendship hospital were collected, and their clinical manifestations and laboratory tests were analyzed retrospectively. The process of acute severe hepatic lesion in 10 AOSD cases was studied carefully. Results Among the 102 inpatient AOSD cases, 54 （52.9%） had hepatic lesion which presented as the increase of serum transaminase and bilirubin, 8 （7.8%） had acute severe hepatic lesion during treatment, 2 patients with acute severe hepatic lesion from other hospitals. They were treated and followed up in our clinic. These 10 patients were all characterized as ALT 〉1000 IU/L, TBil 40-250 mg/L, and mild liver dysfunction at beginning of the disease, but acute severe hepatic lesion occurred due to the use of antibiotics, immunodepressant or NSAIDs. Three patients developed liver function failure and died, 7 patients＇ liver function recovered to normal by increasing dosage of glucocorticoid. Conclusion AOSD with mild liver dysfunction is frequently encountered. During the treatment, acute severe hepatic lesion may occur due to certain drugs. Therefore, any drug used in AOSD with mild liver dysfunction should be cautious. Once acute severe hepatic lesion happens, increases the dosage of glucocorticoid promptly can improve prognosis.