成人斯蒂尔病104例临床和疗效分析

目的 探讨成人斯蒂尔病(AOSD)的临床特点、实验室检查结果及疗效.方法 回顾性分析104例AOSD患者的临床资料,总结临床特征、药物治疗方法和疗效.结果 临床表现:高热100%,皮疹95%,关节痛和(或)关节炎90%,咽痛78%,淋巴结肿大66%,脾脏肿大57%,浆膜炎30%;实验室检查中,中性粒细胞增多98%,肝功能异常62%,红细胞沉降率增快96%,血清铁蛋白升高99%.淋巴结活检者100%为反应性增生.94%应用糖皮质激素,66%患者联合应用了免疫抑制剂;激素用量≥140 mg/d者平均缓解时间缩短,复发率低.结论 不明原因的高热患者,合并皮疹、关节痛,血白细胞和炎症指标升高,排除感染和肿瘤后应考虑AOSD.糖皮质激素和免疫抑制剂是治疗AOSD的最有效药物.

Clinical features and therapeutic response of adult onset Still's disease

Objective To evaluate the clinical manifestations and responses to treatment of adult onset Still's disease (AOSD).Methods One hundred and four AOSD patients were retrospectively analyzed with clinical features and treatment responses.Results The major clinical features were:spiking fever (100%),evanescent maculopapular rash (95%),polyarthralgia (90%),sore throat (78%),lymphadenectasis (66%),hepatosplenomegaly (57%),hydmhymenitis (30%),neutrephilia (98%),liver dysfunction (62%),increased erythrocyte sedimentation rate (96%) and hyperferritinaemia (99%).Reactive lymphnodes hyperplasia was shown in all patients underwent lymphnode biopsy.94% and 66% patients were treated with glucocorticoid and immune suppressants.Those treated with ≥40 mg/d prednisone or its equivalent dosage achieved quicker remission and less relapses.Conclusion Patients who present with spiking fever,polyarthralgia,maculopapular rash,sore throat and predominant neutrophilia should be considered the possibility of AOSD after tumor or infection are excluded.Glucoeorticoid and immune suppressive drugs are effective medications for AOSD.