作者：Ortiz-Sanjuán F 翻译：刘蕊 审校：翟佳羽
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摘要
成人斯蒂尔病（AOSD）往往经常规疗法治疗后复发。阿那白滞素（ANK）是一种白介素-1受体拮抗剂，已有一些个案或小样本研究报道证明其对成人斯蒂尔病有效。
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内容
我们评估了一系列成人斯蒂尔病患者阿那白滞素的疗效。多中心回顾性开放性研究，阿那白滞素用于常规免疫抑制药物无效，甚至一些还应用过至少1种生物制剂的患者。纳入41例患者（26例女性/ 15例男性）。他们平均年龄34.4±14岁，应用阿那白滞素前的病程中位数3.5[2-6]年。当时最常见的临床表现为关节症状87.8％，发热78％，皮疹58.5％。阿那白滞素使得临床症状和实验室指标迅速获得改善并保持。治疗后1年，关节和皮肤症状的发生频率分别下降至41.5％和7.3％，发热从78％降至14.6％，贫血从56.1％降到9.8％，淋巴结肿大从26.8％降到4.9％。实验室参数也取得了显著的改善。泼尼松剂量的中位数[IQR]也从治疗前的20 [11.3-47.5] mg /天减少到阿那白滞素治疗12个月后 5 [0-10]mg /天。随访中位数[IQR]16 [5-50]个月，最主要的副作用是皮肤表现（8例）、轻度白细胞减少症（3例）、肌病（1例）和感染（5例）。阿那白滞素与快速而持久的临床和实验室的改善相关，甚至对其他生物制剂无反应的患者也包括在内。然而，关节表现是比全身症状更不易控制。
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附原文
Adult-onset Still's disease (AOSD) is often refractory to standard therapy. Anakinra (ANK), an interleukin-1 receptor antagonist, has demonstrated efficacy in single cases and small series of AOSD. We assessed the efficacy of ANK in a series of AOSD patients. Multicenter retrospective open-label study. ANK was used due to lack of efficacy to standard synthetic immunosuppressive drugs and in some cases also to at least 1 biologic agent. Forty-one patients (26?women/15 men) were recruited. They had a mean age of 34.4?±?14 years and a median [interquartile range (IQR)] AOSD duration of 3.5 [2-6] years before ANK onset. At that time the most common clinical features were joint manifestations 87.8%, fever 78%, and cutaneous rash 58.5%. ANK yielded rapid and maintained clinical and laboratory improvement. After 1 year of therapy, the frequency of joint and cutaneous manifestations had decreased to 41.5% and to 7.3% respectively, fever from 78% to 14.6%, anemia from 56.1% to 9.8%, and lymphadenopathy from 26.8% to 4.9%. A dramatic improvement of laboratory parameters was also achieved. The median [IQR] prednisone dose was also reduced from 20 [11.3-47.5] mg/day at ANK onset to 5 [0-10] at 12 months. After a median [IQR] follow-up of 16 [5-50] months, the most important side effects were cutaneous manifestations (n?=?8), mild leukopenia (n?=?3), myopathy (n?=?1), and infections (n?=?5).ANK is associated with rapid and maintained clinical and laboratory improvement, even in nonresponders to other biologic agents. However, joint manifestations are more refractory than the systemic manifestations.
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引自
Ortiz-Sanjuán F, Blanco R, Riancho-Zarrabeitia L, et al.Efficacy of Anakinra in Refractory Adult-Onset Still's Disease: Multicenter Study of 41 Patients and Literature Review. Medicine (Baltimore). 2015 Sep;94(39):e1554. doi: 10.1097/MD.0000000000001554.
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