【摘要】 目的探讨儿童和成人斯蒂尔病的临床特征、诊断、治疗和转归。方法回顾分析确诊的44例儿童斯蒂尔病(sJIA)和112例成人斯蒂尔病(AOSD)的临床表现、实验室检查、治疗和预后。结果AOSD和sJIA在病程和男女发病比例上差异无统计学意义。发热、关节痛和皮疹是二者最常见的症状。成人患者咽痛、皮疹、肌痛、肺组织受累、胸膜病变比例高于儿童,儿童肝肿大比例高于成人。白细胞增多见于大部分患者,儿童血小板增多比例高于成人,而血清铁蛋白升高率低于成人。AOSD和sJIA在治疗方案选择和病情转归方面差异无统计学意义。非甾体消炎药(NSAID)对某些轻中度的患者有一定效果,但多数情况下需合用激素,甚至免疫抑制剂,其中首选甲氨蝶呤。结论AOSD和sJIA均是以发热、皮疹、关节痛和白细胞增多为主要表现的自身免疫性疾病,预后良好,但部分患者可反复发作或呈慢性持续性。多数患者对激素治疗有效,但对以关节炎表现为主以及合并内脏损害的患者应尽早加用免疫抑制剂,以控制病情、减少复发和关节畸形。更多还原

【Abstract】 Objective To compare the clinical and laboratory features, disease course, treatment response and prognosis between adult onset Still′s disease (AOSD) and systemic juvenile idiopathic arthritis (sJIA). Methods We retrospectively reviewed all available data from patients with a confirmed diagnosis of AOSD (n=44) or sJIA (n=112). Results There were no significant differences in the disease duration and gender distribution between the AOSD and sJIA cases. Fever, rash and arthritis were the most common symptoms in both conditions. The frequencies of sore throat, rash, myalgia, pulmonary involvement and pleural effusion were higher in AOSD, while hepatomegaly was more frequent in sJIA. Laboratory findings showed leukocytosis in majority of the patients, but more sJIA patients were found to have thrombocytosis, and more AOSD cases were found to be associated with increased serum ferritin. There were no significant differences in the treatment options and prognosis between AOSD and sJIA. Non-steroid anti-inflammatory drugs can be effective in mild to moderate cases, but most frequently, combined use of corticosteroids and even immunosuppressants may be indicated, of which, methotrexate should be considered as the first-line agent. Conclusion AOSD and sJIA are both systemic autoimmune diseases characterized by daily febrile spikes, evanescent rash, arthritis and leukocytosis due to unknown causes. Prognosis of these conditions is generally good, but in some cases, symptoms may be relapsing or long-lasting. Most patients respond well to steroid therapy, however, in those with arthritis-predominant signs or complicating damage to organs, immunosuppressants may be needed.更多还原