背景:成人发作的斯蒂尔病(AOSD)是一种罕见的以发烧为特征的疾病,皮疹,和关节痛/关节炎。在荷兰,大多数治疗AOSD的医生每年治疗<5名患者。目前,没有国际公认的AOSD治疗指南。
目的:进行Delphi小组,旨在就AOSD患者的诊断和治疗策略达成共识,并将结果作为治疗算法的基础。
方法:Delphi小组汇集了18位AOSD专家:风湿病学家,内科医生和儿科医生。Delphi过程由3轮组成。在前两轮中,在线问题和陈述列表已完成。在第三轮中,在虚拟会议上讨论了最后声明,并进行了最终投票。共识门槛设定为80%。进行了两次有针对性的文献检索,以确定基于共识的陈述的证据水平。
结果:就29项声明达成共识,包括与诊断和诊断测试有关的陈述,反应和缓解的定义,治疗,使用甲氨蝶呤,逐渐减少治疗。专家组同意减少糖皮质激素的使用以避免副作用,与常规治疗相比,更喜欢使用生物制剂。白细胞介素-1和白细胞介素-6阻断剂的作用被认为在AOSD的治疗中很重要。
结论:在此Delphi面板中,对于AOSD的诊断和治疗建议达成了高度共识,可作为治疗指南的基础.
OBJECTIVE: Adult-onset Still\'s disease (AOSD) is a rare condition characterized by fevers, rash, and arthralgia/arthritis; most doctors treating AOSD in the Netherlands treat <5 patients per year. Currently, there is no internationally accepted treatment
guideline for AOSD. The objectives of this study were to conduct a Delphi panel aimed at reaching
consensus about diagnostic and treatment strategies for patients with AOSD and to use the outcomes as a basis for a treatment algorithm.
METHODS: The Delphi panel brought together 18 AOSD experts: rheumatologists, internists and paediatricians. The Delphi process consisted of three rounds. In the first two rounds, online lists of questions and statements were completed. In the third round, final statements were discussed during a virtual meeting and a final vote took place.
Consensus threshold was set at 80%. Two targeted literature searches were performed identifying the level of evidence of the consensus-based statements.
RESULTS: Consensus was reached on 29 statements, including statements related to diagnosis and diagnostic tests, definition of response and remission, the therapy, the use of methotrexate and tapering of treatment. The panel consented on reduction of the use of glucocorticoids to avoid side effects, and preferred the use of biologics over conventional treatment. The role of IL-1 and IL-6 blocking agents was considered important in the treatment of AOSD.
CONCLUSIONS: In this Delphi panel, a high level of consensus was achieved on recommendations for diagnosis and therapy of AOSD that can serve as a basis for a treatment
guideline.