成人斯蒂爾?。ˋOSD）往往經(jīng)常規(guī)療法治療后復(fù)發(fā)。阿那白滯素（ANK）是一種白介素-1受體拮抗劑，已有一些個(gè)案或小樣本研究報(bào)道證明其對成人斯蒂爾病有效。

我們評估了一系列成人斯蒂爾病患者阿那白滯素的療效。多中心回顧性開放性研究，阿那白滯素用于常規(guī)免疫抑制藥物無效，甚至一些還應(yīng)用過至少1種生物制劑的患者。納入41例患者（26例女性/ 15例男性）。他們平均年齡34.4±14歲，應(yīng)用阿那白滯素前的病程中位數(shù)3.5[2-6]年。當(dāng)時(shí)最常見的臨床表現(xiàn)為關(guān)節(jié)癥狀87.8％，發(fā)熱78％，皮疹58.5％。阿那白滯素使得臨床癥狀和實(shí)驗(yàn)室指標(biāo)迅速獲得改善并保持。治療后1年，關(guān)節(jié)和皮膚癥狀的發(fā)生頻率分別下降至41.5％和7.3％，發(fā)熱從78％降至14.6％，貧血從56.1％降到9.8％，淋巴結(jié)腫大從26.8％降到4.9％。實(shí)驗(yàn)室參數(shù)也取得了顯著的改善。潑尼松劑量的中位數(shù)[IQR]也從治療前的20 [11.3-47.5] mg /天減少到阿那白滯素治療12個(gè)月后 5 [0-10]mg /天。隨訪中位數(shù)[IQR]16 [5-50]個(gè)月，最主要的副作用是皮膚表現(xiàn)（8例）、輕度白細(xì)胞減少癥（3例）、肌病（1例）和感染（5例）。阿那白滯素與快速而持久的臨床和實(shí)驗(yàn)室的改善相關(guān)，甚至對其他生物制劑無反應(yīng)的患者也包括在內(nèi)。然而，關(guān)節(jié)表現(xiàn)是比全身癥狀更不易控制。

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.

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.