Adult-onset Still disease
Adult-onset Still disease
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DOI：
10.1016/j.berh.2008.08.006
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发表时间：
2008-10-01
影响因子：
5.2
通讯作者：
Fautrel, Bruno
中科院分区：
文献类型：
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作者：
Fautrel, Bruno
成人斯蒂尔病（AOSD）是一种病因不明的罕见炎症性疾病，通常有四个主要症状：体温≥ 39 ℃，关节痛或关节炎，皮疹和白细胞增多症（≥ 10，000个细胞/mm 3），中性粒细胞≥ 80%。由于许多其他表现是可能的，诊断是潜在的挑战。测定总铁蛋白和糖化铁蛋白水平，虽然不是特异性的，但有助于诊断。AOSD的疾病演变可以是单周期、多周期或慢性的。在慢性疾病中，关节受累通常占主导地位，三分之一的患者会出现糜烂。迄今为止尚未发现任何预后因素。治疗策略来自观察数据。皮质类固醇通常是一线治疗。由于对皮质类固醇的反应不足，甲氨蝶呤似乎是控制疾病活动和减少类固醇使用的最佳选择。对于难治性疾病，使用阻断白细胞介素-1（阿那白滞素）的药物进行生物治疗，然后使用阻断白细胞介素-6（托珠单抗）的药物进行生物治疗似乎是最有希望的。
Adult-onset Still disease (AOSD) is an uncommon inflammatory condition of unknown origin typically characterized by four main (cardinal) symptoms: spiking fever >= 39 degrees C, arthralgia or arthritis, skin rash and hyperleucocytosis (>= 10,000 cells/mm(3)) with neutrophils >= 80%. As many other manifestations are possible, diagnosis is potentially challenging. Determination of the total and glycosylated ferritin levels, although not pathognomonic, can help in diagnosis. The disease evolution of AOSD can be monocyclic, polycyclic or chronic. In chronic disease, joint involvement is often predominant and erosions are noted in one-third of patients. No prognostic factors have been identified to date. Therapeutic strategies are from observational data. Corticosteroids are usually the first-line treatment. With inadequate response to corticosteroids, methotrexate appears the best choice to control disease activity and allow for tapering of steroid use. For refractory disease, biological therapy with agents blocking interleukin-1 (anakinra) and then those blocking interleukin-6 (tocilizumab) seem the most promising.