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张莉芸

张莉芸

主任医师 教授
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医学博文

阿那白滞素可治疗难治性成人斯蒂尔病

发表者:张莉芸 1748人已读

翻译:刘樱  修改:庞宇洁  

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。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。山西白求恩医院风湿免疫科张莉芸

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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发表于:2017-09-11 15:51

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张莉芸的咨询范围: 类风湿关节炎、强直性脊柱炎和系统性红斑狼疮的早期诊断和治疗;干燥综合征、皮肌炎、多发性肌炎、硬皮病、白塞病、复发性多软骨炎、痛风、骨关节炎、骨质疏松症等的诊断治疗;各型血管炎(韦格纳肉芽肿、显微镜下多血管炎、变应性肉芽肿性血管炎、过敏性紫癜等)的诊断和治疗;发热的诊断和鉴别诊断;应用间充质干细胞治疗重症狼疮、血管炎、类风湿关节炎等弥漫性结缔组织病。 更多>>