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Update on the management of colchicine resistant Familial Mediterranean Fever (FMF).
Orphanet Journal of Rare Diseases ( IF 3.4 ) Pub Date : 2019-10-15 , DOI: 10.1186/s13023-019-1201-7
Georges El Hasbani 1 , Ali Jawad 2 , Imad Uthman 1
Affiliation  

BACKGROUND Familial Mediterranean Fever (FMF), an autoinflammatory disease, is characterized by self-limited inflammatory attacks of fever and polyserositis along with high acute phase response. Although colchicine remains the mainstay in treatment, intolerance and resistance in a certain portion of patients have been posing a problem for physicians. MAIN BODY Like many autoimmune and autoinflammatory diseases, many colchicine-resistant or intolerant FMF cases have been successfully treated with biologics. In addition, many studies have tested the efficacy of biologics in treating FMF manifestations. CONCLUSION Since carriers of FMF show significantly elevated levels of serum TNF alpha, IL-1, and IL-6, FMF patients who failed colchicine were successfully treated with anti IL-1, anti IL-6, or TNF inhibitors drugs. It is best to use colchicine in combination with biologics.

中文翻译:

秋水仙碱抗性家族性地中海热(FMF)的管理更新。

背景技术家族性地中海热(FMF)是一种自发性疾病,其特征在于发烧和多发性浆膜炎的自限性炎症发作以及高度急性期反应。尽管秋水仙碱仍然是治疗的主要手段,但某些患者的不耐受和耐药性已成为医师的难题。与许多自身免疫性疾病和自身炎症性疾病一样,许多对秋水仙碱耐药或不耐受的FMF病例已通过生物制剂成功治疗。此外,许多研究已经测试了生物制剂治疗FMF表现的功效。结论由于FMF携带者的血清TNFα,IL-1和IL-6水平显着升高,因此秋水仙碱失败的FMF患者已成功接受抗IL-1,抗IL-6或TNF抑制剂药物的治疗。
更新日期:2019-10-15
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