成人still病回顾性分析

RETROSPECTIVE ANALYSIS OF ADULT ONSET STILL'S DISEASE

目的:探讨成人Still病(AOSD)的临床特点和治疗,提高对该病的认识。方法:回顾性分析从2002年1月至2008年3月在本院住院诊断的58例AOSD患者的临床特点、诊断、治疗和预后。结果:临床表现以发热(100.00%)、关节症状(93.10%)、皮疹(82.76%)为主,次要表现有咽痛(67.24%)、脾肿大(65.52%)、肌痛(48.28%)、淋巴结肿大(41.38%)、肝肿大(34.48%)、腹泻腹痛(6.70%);辅助检查:白细胞增高(100.00%),血红蛋白下降(62.07%),血小板升高(87.93%),肝功能异常(67.24%),血沉增快(100.00%),C反应蛋白升高(100.00%),血清铁蛋白升高(87.93%),骨髓检查异常(89.47%)。治疗:糖皮质激素治疗有效,大部分患者需同时联合应用免疫抑制剂,预后良好。结论:成人Still病临床表现复杂多样,常多系统受累,血清铁蛋白升高对诊断有帮助,糖皮质激素治疗有效,大部分患者需联合应用免疫抑制剂,有必要进行长期随访。

Objective： To study the clinical features ,diagnosis, treatment and outcome of patients with Adult Onset Still＇s disease （AOSD）. Methods： A retrospective analysis of 58 patients with AOSD hospitalized from 2002 to 2008 at our Hospital. Their clinical and laboratory features, disease course , treatment , and outcomes were analyzed . Results： The most common clinical manifestations were fever （100 %） , articular symptoms （93.10 %） and rash （82.76 %） .Sore throat （67.24 %） , splenomegaly （65.52 %） ,myalgia （48.28 %） , lymphadenectasis （41.38 %）, hepatomegaly （34.48 %）, diarrhea （6.70 %） were also involved .All patients had elevation of leuco- cyte ,erythroeyte sedimentation rate and C-reactive protein .Hyperferritinemia and thromboeytosis were present in 87.93 % of cases .Hepatic enzymes were elevated in 67.24 % of cases . Bone marrow was abnormal in 89.47 % of cases . 62,07 % of cases were Anemic . It is a relatively benign disease if diagnosed early and treated appropriately . Glucocorticoid is often effective , but immunosuppressive may be needed in most patients . Conclusion：AOSD wasa complicated and diverse disease with multiple systems affected ; hyperferritinemia was helpful to diagnosis . Glucocorticoid is often effective ,but immunosuppressive may be needed in most patients. Longterm follow-up is important.