Department of Allergy and Rheumatology, and BK21 Division of Cell Transformation and Restoration, Ajou University School of Medicine, Woncheon-dong San 5, Youngtong-gu, Suwon 443-721, Korea.
Rheumatol Int. 2012 May;32(5):1291-8. doi: 10.1007/s00296-011-1801-6. Epub 2011 Jan 29.
To date, the treatment of adult-onset Still's disease (AOSD) has been largely empirical; therefore, this study was conducted to investigate the response to therapy and prognostic factors of AOSD. Fifty-four Korean patients with AOSD were enrolled based on Yamaguchi's criteria. We retrospectively analyzed the treatments and prognosis. Thirty-nine patients (72.2%) were female, and the average age at disease onset was 37.3 years. Twenty-nine patients had a monocyclic disease (53.7%), five had a polycyclic (9.3%) and fifteen had a chronic articular disease (27.7%) and five died (9.3%). The elevated ESR and corticosteroids refractoriness were associated with poor prognosis (P = 0.023 and P = 0.009, respectively). The patients that died were older than those survived (49.2 ± 11.8 vs. 42.2 ± 14 year old, P = 0.024). Forty-two patients were treated with non-steroidal anti-inflammatory drugs; however, they also needed corticosteroids and intravenous immunoglobulin (IVIG). Among 50 patients treated with high-dose corticosteroids, 21 patients (42%) were resistant to corticosteroids and treated with IVIG or anti-tumor necrosis factor (TNF) agents. Of the 23 patients medicated with IVIG, the prognosis was better in IVIG-responsive patients, indicating a therapeutic effect. Methotrexate was the most commonly used disease modifying anti-rheumatic drugs (27 patients, 50%), and the corticosteroid requirements were lower in the methotrexate-responsive patients. Approximately half of AOSD patients had a poor prognosis and were corticosteroids resistance. An elevated ESR and non-response to corticosteroids were associated with poor prognosis. Patients who died were older than those survived.
迄今为止，成人Still 病（AOSD）的治疗主要是经验性的；因此，本研究旨在探讨 AOSD 的治疗反应和预后因素。根据 Yamaguchi 的标准，我们共纳入了 54 例韩国 AOSD 患者。我们回顾性分析了这些患者的治疗和预后情况。39 例（72.2%）为女性，发病平均年龄为 37.3 岁。29 例为单环型疾病（53.7%），5 例为多环型（9.3%），15 例为慢性关节疾病（27.7%），5 例死亡（9.3%）。升高的 ESR 和皮质类固醇激素抵抗与不良预后相关（P = 0.023 和 P = 0.009）。死亡患者的年龄大于存活患者（49.2 ± 11.8 岁 vs. 42.2 ± 14 岁，P = 0.024）。42 例患者接受了非甾体抗炎药治疗；然而，他们也需要皮质类固醇激素和静脉注射免疫球蛋白（IVIG）。在 50 例接受大剂量皮质类固醇激素治疗的患者中，有 21 例（42%）对皮质类固醇激素耐药，接受了 IVIG 或抗肿瘤坏死因子（TNF）药物治疗。在接受 IVIG 治疗的 23 例患者中，IVIG 反应良好的患者预后较好，表明治疗有效。甲氨蝶呤是最常用的改善病情抗风湿药物（27 例，50%），且在甲氨蝶呤反应良好的患者中，皮质类固醇激素的需求较低。大约一半的 AOSD 患者预后不良且对皮质类固醇激素耐药。升高的 ESR 和对皮质类固醇激素无反应与不良预后相关。死亡患者的年龄大于存活患者。
