Familial Mediterranean fever is characterized by self-limited attacks of serositis and arthritis. However, substantial number of patients suffer from chronic complications of this disease, primarily involving musculoskeletal system. Treatment for these complications is challenging due to limited evidence. Interleukin-1 (IL-1) antagonists, tocilizumab and anti-tumor necrosis factor (anti-TNF) agents are off-label treatment options for the management of chronic manifestations of FMF, such as secondary (AA) amyloidosis, chronic arthritis and sacroiliitis. This paper presents a case series of four FMF patients who are refractory to IL-1 antagonists, anti-TNF agents and tocilizumab, who responded well to tofacitinib. The authors also conducted a comprehensive literature search for studies investigating tofacitinib use in FMF patients. Although still limited, current data suggest that tofacitinib could be a useful treatment option for FMF patients with associated inflammatory comorbid conditions and chronic manifestations of disease.

中文翻译：

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托法替尼治疗耐秋水仙碱的家族性地中海热：基于病例的审查。

家族性地中海热的特征是浆膜炎和关节炎的自限发作。然而，相当多的患者患有该疾病的慢性并发症，主要涉及肌肉骨骼系统。由于证据有限，这些并发症的治疗具有挑战性。白介素-1（IL-1）拮抗剂，托珠单抗和抗肿瘤坏死因子（anti-TNF）药物是用于管理FMF慢性表现（如继发性（AA）淀粉样变性病，慢性关节炎和sa肌炎）的非标治疗选择。本文介绍了四例FMF患者，这些患者对IL-1拮抗剂，抗TNF药物和tocilizumab耐药，对tofacitinib的反应良好。作者还进行了全面的文献检索，以研究托法替尼在FMF患者中的使用情况。