摘要目的 探讨成人斯蒂尔病(AOSD)的临床、免疫学特征、临床疗效及预后相关因素.方法 回顾性分析137例AOSD患者的临床特征、免疫学特点,随访患者病情缓解、复发及转归情况.组间率的比较采用x2检验,采用Cox回归分析与病情复发的相关因素.结果 发热、皮疹、关节炎为AOSD的三大主征.实验室检查以血象异常及炎性指标尤其是铁蛋白升高为主(128例,97.1％),肝酶升高多见.共随访98例患者,药物治疗4周总缓解率为74％(73例),糖皮质激素联合改善病情抗风湿药治疗缓解率显著高于单用糖皮质激素,以糖皮质激素联合甲氨蝶呤及羟氯喹缓解率最高(8/8),其次为糖皮质激素联合甲氨蝶呤( 25/28,89％)及糖皮质激素联合羟氯喹(14/16,88％).41例(42％)患者疾病复发,其中单用糖皮质激素的复发率为61％(23/38),显著高于糖皮质激素联合改善病情抗风湿药组(29％,P=0.004).Cox回归分析发现加用甲氨蝶呤有助于减少疾病复发[(RR=0.418,95％CI (0.192～0.909),P=0.028].5例(5％)患者在随访中确诊为其他疾病.结论 起始足量糖皮质激素联合改善病情抗风湿药治疗有利于AOSD疾病缓解,减少复发；加用甲氨蝶呤可减少疾病复发.

abstractsObjective To investigate the clinical and immunological features,therapeutic response as well as prognosis of adult onset Still's disease (AOSD).Methods AOSD was diagnosed in 137 patients referred to our department.Clinical and immunological data were retrospectively analyzed.Therapeutic response and prognosis were systemically reviewed during the follow-up period.Intergroup incidence divergence was analyzed by chi-square test.Cox regression analysis was adopted to determine factors related with relapse.Results Fever,rash and arthritis were the cardinal clinical features of AOSD patients.Elevated inflammatory indices including ferritin (128 suhjects,97.1％ ) along with neutrophilia and liver dysfunction were the main laboratory findings.Ninety-eight patients were followed up and 75％ (73 subjects) had achieved complete remission after 4 weeks treatment.Forty-one patients (42％) who had achieved remission relapsed during follow-up period.Combination of glucocorticoid steroid and disease modifying antirheumatic drugs (DMARDs) were more effective than glucocorticoid steroid only in inducing remission and preventing relapse.More patients received glucocorticoid combined with methotrcxate and hydroxychloroquine achieved remission (8 of 8 patients) than patients who were treated with glucocorticoid and methotrexate (25 of 28 patients,89％ ) and those treated with glucocorticoid and hydroxychloroquine (14 of 16 patients,88％ ).Patients with glucocorticoid were more likely to suffer disease recurrence than those who took glucocorticoid combined with DMARDs (61％ vs 29％,P=0.004).Cox regression analysis suggested that methotrexate had protec-tive effect against recurrence [RR=0.418,95％CI (0.192-0.909),P=0.028].5％ of patients were diagnosed to other diseases during the follow up period.Conclusion Initial treatment with combined glucocorticoid and DMARDs is beneficial to induce remission and prevent reoccurrence.Methotrcxate has a protective effect against recurrence.