成人发作的斯蒂尔病(AOSD)是一种罕见的炎症,以发烧为特征,皮疹,和关节炎。因为它的稀有性,临床试验本来就很小,而且往往不受控制.我们的目标是为使用白介素(IL)-1抑制剂治疗AOSD患者提供建议。基于最好的证据和专家意见。
成立了一个由10名专家(9名风湿病学家和1名儿科医生)组成的小组。第一步是致力于全面的文献回顾和陈述的发展。在MEDLINE(Pubmed)上进行了两次单独的文献检索,EMBASE,和截至2018年4月的BIOSIS数据库,以确定(1)AOSD与小儿斯蒂尔病(系统性幼年特发性关节炎[SJIA])之间的差异和相似性,以及(2)IL-1抑制剂在AOSD治疗中的疗效和安全性。第二步,这些陈述以Delphi程序提交给一个由67名风湿病学家组成的小组.共识阈值设定为66%:积极,>66%的选民选择了3到5分;负面的,>66%的选民选择得分1或2。第三步,对投票结果进行了分析,声明已经定稿。
共发表了11项声明。67位风湿病专家中有46位(72%)参与了Delphi过程。在第一轮投票后达成了积极的共识,并且在大多数声明中获得了充分的共识(>95%)。在将AOSD和SJIA视为同一疾病方面达成了广泛共识。在难治性患者中使用抗IL-1治疗被认为是非常安全和有效的,作为第一个和随后的生物治疗线。尤其是全身患者。由于缺乏正面比较,无法确定IL-1抑制剂之间的不同疗效。有一个很大的共识,即第一种IL-1抑制剂的失败并不排除对另一种IL-1抑制剂的反应。缺乏比较早期和晚期治疗的研究不能得出结论;然而,来自SJIA的数据表明在早期治疗中有更好的反应。
Delphi方法用于制定建议,我们希望这些建议将有助于临床医生对常规疗法难治性AOSD患者的管理。
Adult-onset Still\'s disease (AOSD) is a rare inflammatory condition characterized by fever, rash, and arthritis. Because of its rarity, clinical trials are inherently small and often uncontrolled. Our objective was to develop recommendations for the use of interleukin (IL)-1 inhibitors in the management of patients with AOSD, based on the best evidence and expert opinion.
A panel of 10 experts (9 rheumatologists and 1 pediatrician) was established. The first step was dedicated to a comprehensive literature review and development of statements. Two separate literature searches were performed on the MEDLINE (Pubmed), EMBASE, and BIOSIS databases through April 2018 to identify (1) differences and similarities between AOSD and pediatric Still\'s disease (systemic juvenile idiopathic arthritis [SJIA]) and (2) the efficacy and safety of IL-1 inhibitors in AOSD treatment. In the second step, the statements were submitted in a Delphi process to a panel of 67 rheumatologists.
Consensus threshold was set at 66%: positive, > 66% of voters selected scores 3 to 5; negative, > 66% of voters selected scores 1 or 2. In the third step, the voting results were analyzed, and the statements were finalized.
Eleven statements were developed. Forty-six of 67 rheumatologists (72%) participated in the Delphi process. A positive
consensus was reached after the first round of voting and was full (> 95%) on the majority of statements. A large
consensus was achieved in considering AOSD and SJIA as the same disease. The use of anti-IL-1 therapies in refractory patients was considered quite safe and effective both as the first and as a subsequent line of biologic treatment, especially in systemic patients. Because of the lack of head-to-head comparisons, a different profile of efficacy among IL-1 inhibitors could not be established. There was a large
consensus that failure of the first IL-1 inhibitor does not preclude response to another one. The lack of studies comparing early versus late treatment did not allow to draw conclusions; however, data from SJIA suggest a better response in early treatment.
The Delphi method was used to develop recommendations that we hope will help clinicians in the management of patients with AOSD refractory to conventional therapies.