Mok C C, Lau C S, Wong R W
Department of Medicine, Queen Mary Hospital, Pokfulam, Hong Kong.
J Rheumatol. 1998 Dec;25(12):2345-51.
To study the clinical characteristics, treatment outcome, and complications of patients with adult onset Still's disease (AOSD) in our local Chinese population.
Patients with AOSD were identified among others who attended our rheumatology clinics from 1967 to 1997 and were followed. Their clinical and laboratory features at presentation, treatment, and outcome were recorded and compared with other reported series.
Sixteen patients with AOSD were identified. Eleven (69%) were female. Nine (56%) had onset of the disease between 16 and 35 years of age. The commonest presenting features were fever (100%), arthritis (94%), rash (85%), weight loss (69%), and sore throat (63%). Fifteen patients presented with pyrexia of unknown origin and the median duration of fever before the establishment of the diagnosis was 6 weeks (range 4-75). The acute phase response was marked in all patients with gross elevation of erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and complement levels. Hyperferritinemia (> 5 times normal) was present in 90% of cases. Most patients (81%) required corticosteroid therapy and 85% of those steroid treated patients received additional disease modifying agents. The mean duration of followup of our patients was 93.3 months (range 8-362). Five (33%) had monocyclic systemic disease, 6 (40%) had polycyclic systemic disease, and 4 (27%) had frequent relapses that progressed to a chronic arthropathy.
AOSD in southern Chinese tends to run a benign course, with few patients evolving into chronic inflammatory arthropathy. A significantly lower incidence of serositis, lung involvement, and enlargement of the reticuloendothelial organs was observed at presentation compared with patients of different ethnic origins.
研究我国本地汉族人群中成人斯蒂尔病（AOSD）患者的临床特征、治疗效果及并发症。
在1967年至1997年期间到我们风湿科门诊就诊的患者中识别出AOSD患者并进行随访。记录他们就诊时、治疗过程及结局的临床和实验室特征，并与其他报道的系列进行比较。
共识别出16例AOSD患者。11例（69%）为女性。9例（56%）发病年龄在16至35岁之间。最常见的临床表现为发热（100%）、关节炎（94%）、皮疹（85%）、体重减轻（69%）和咽痛（63%）。15例患者以不明原因发热就诊，确诊前发热的中位持续时间为6周（范围4 - 75周）。所有患者急性期反应均明显，红细胞沉降率（ESR）、C反应蛋白（CRP）和补体水平显著升高。90%的病例存在高铁蛋白血症（>正常上限5倍）。大多数患者（81%）需要糖皮质激素治疗，其中85%接受糖皮质激素治疗的患者还加用了改善病情的药物。我们患者的平均随访时间为93.3个月（范围8 - 362个月）。5例（33%）为单循环系统性疾病，6例（40%）为多循环系统性疾病，4例（27%）频繁复发并进展为慢性关节炎。
中国南方的AOSD往往病程良性，很少有患者发展为慢性炎症性关节炎。与不同种族的患者相比，就诊时浆膜炎、肺部受累及网状内皮器官肿大的发生率明显较低。
