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成人斯蒂尔病患者治疗目标达到早期及长期缓解的专家共识。

Expert consensus on the treatment of patients with adult-onset still's disease with the goal of achieving an early and long-term remission.

机构信息

U.O.C. di Immunoreumatologia, Policlinico Universitario Campus Bio-Medico, Rome, Italy; Research Unit of Immunorheumatology, Department of Medicine and Surgery, Università Campus Bio-Medico di Roma, Rome, Italy.

Dipartimento di Reumatologia e Scienze Mediche - ASST Gaetano Pini-CTO, Milan; Università degli studi di Milano, Milano, Italy.

出版信息

Autoimmun Rev. 2023 Dec;22(12):103400. doi: 10.1016/j.autrev.2023.103400. Epub 2023 Jul 22.

Abstract

We performed a comprehensive systematic targeted literature review and used the Delphi method to formulate expert consensus statements to guide the treatment of adult-onset Still's disease (AOSD) to achieve an early and long-term remission. Seven candidate statements were generated and reached consensus in the first round of voting by the panel of experts. We postulate: (i) In patients with AOSD with predominant arthritis at onset who achieved no disease control with glucocorticoids (GCs), the use of methotrexate can be considered, whereas the use of cyclosporin A and low-dose GCs should not (Statements 1-3); (ii) In patients with AOSD with poor prognostic factors at diagnosis, an IL-1 inhibitor (IL-1i) in addition to GCs should be taken into consideration as early as possible (Statement 4); (iii) A switch to an IL-6 inhibitor (IL-6i) may be considered in patients with AOSD with prevalent joint involvement, who are unresponsive or intolerant to IL-1i (Statement 5); (iv) Drug tapering or discontinuation may be considered in patients who achieved a sustained clinical and laboratory remission with IL-1i (Statement 6); (v) In patients with AOSD who failed to attain a good clinical response with an IL-1i, switching to an IL-6i may be considered in alternative to a different IL-1i. TNF-inhibitors may be considered as a further choice in patients with a prominent joint involvement (Statement 7). These statements will help clinicians in treatment decision making in patients with AOSD.

摘要

我们进行了全面的系统靶向文献综述,并使用德尔菲法制定专家共识声明,以指导成人斯蒂尔病(AOSD)的治疗,实现早期和长期缓解。提出了 7 个候选声明,并在专家组的第一轮投票中达成共识。我们假设:(i)在以关节炎为主要表现且糖皮质激素(GCs)治疗未控制疾病的 AOSD 患者中,可以考虑使用甲氨蝶呤,而不应使用环孢素 A 和低剂量 GCs(声明 1-3);(ii)在诊断时具有不良预后因素的 AOSD 患者中,应尽早考虑除 GCs 外还使用白细胞介素-1 抑制剂(IL-1i)(声明 4);(iii)对于关节受累为主且对 IL-1i 无反应或不耐受的 AOSD 患者,可考虑换用白细胞介素-6 抑制剂(IL-6i)(声明 5);(iv)对于使用 IL-1i 达到持续临床和实验室缓解的患者,可考虑逐渐减少或停用药物(声明 6);(v)对于未对 IL-1i 产生良好临床反应的 AOSD 患者,可考虑换用 IL-6i,而不是不同的 IL-1i(声明 7)。对于关节受累明显的患者,TNF 抑制剂也可作为进一步的选择。这些声明将有助于临床医生在 AOSD 患者的治疗决策中。

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