Zhu Guihua, Liu Gang, Liu Yixin, Xie Qibing, Shi Guixiu
Department of Immunology, West China Medical School, Sichuan University, Chengdu, China.
J Clin Rheumatol. 2009 Sep;15(6):284-8. doi: 10.1097/RHU.0b013e3181b57199.
Adult-onset Still's disease (AOSD) is a systemic inflammatory disorder of unknown etiology, characterized by high fever, transient cutaneous rash, arthralgia/arthritis, and leukocytosis. Liver involvement in AOSD has been described, but few reports have described it in depth. The present study analyzed clinical and laboratory features in a series of Chinese AOSD patients.
Data of 77 patients with AOSD (fulfilling Yamaguchi's diagnostic criteria) were retrospectively reviewed and compared with other series.
The characteristics of our patients are similar to those reported in the literature. Hepatomegaly occurred in 11.7% of the cases; abnormal liver enzymes in 62.3% mild cytolysis (level of transaminases <2 N) (23.4%), moderate cytolysis (between 2 and 5 N) (23.4%), severe cytolysis (>5 N) (15.6%), and increase in the level of alkaline phosphatase (32.9%), gamma-glutamyltransferase (48.1%), lactic dehydrogenase (69.0%). Complete recovery occurred in all patients, except for 1 who died of severe liver failure and complications.
AOSD is a systemic disease, and the present study reemphasizes the high frequency of liver involvement. Although it was slight to moderate in most cases, severe cytolytic hepatitis has been described. Treatment for AOSD patients with liver involvement aimed mainly at AOSD itself and most of the patients with liver involvement got complete remission with systemic corticosteroid therapy.
成人斯蒂尔病（AOSD）是一种病因不明的全身性炎症性疾病，其特征为高热、一过性皮疹、关节痛/关节炎和白细胞增多。已有关于AOSD肝脏受累的报道，但深入描述的较少。本研究分析了一系列中国AOSD患者的临床和实验室特征。
回顾性分析77例符合山口诊断标准的AOSD患者的数据，并与其他系列研究进行比较。
我们患者的特征与文献报道相似。11.7%的病例出现肝肿大；62.3%的患者肝酶异常，轻度细胞溶解（转氨酶水平<2N）（23.4%），中度细胞溶解（2至5N之间）（23.4%），重度细胞溶解（>5N）（15.6%），碱性磷酸酶水平升高（32.9%），γ-谷氨酰转移酶（48.1%），乳酸脱氢酶（69.0%）。除1例死于严重肝功能衰竭及并发症外，所有患者均完全康复。
AOSD是一种全身性疾病，本研究再次强调了肝脏受累的高发生率。虽然大多数病例为轻度至中度，但也有重度细胞溶解性肝炎的描述。AOSD合并肝脏受累患者的治疗主要针对AOSD本身，大多数肝脏受累患者经全身糖皮质激素治疗后完全缓解。
