Ozdogan Huri, Ugurlu Serdal
a Division of Rheumatology, Department of Internal Medicine , Cerrahpasa Medical Faculty, Istanbul University , Fatih , Istanbul , Turkey.
Expert Rev Clin Immunol. 2017 May;13(5):393-404. doi: 10.1080/1744666X.2017.1313116. Epub 2017 Apr 10.
Familial Mediterranean fever (FMF) is the most frequent of all hereditary autoinflammatory syndromes. It is characterized by recurrent attacks of fever and serositis. If not treated it may be complicated with AA amyloidosis. It is caused by mutations in the MEFV gene that encodes pyrin which is involved in the regulation of IL-1β. The mainstay of treatment is colchicine, however a subset of patients requires an alternative treatment either due to inadequate response or intolerance. The accumulating data indicates that anti IL-1 drugs are effective in treating colchicine resistant FMF cases and improving their quality of life. Areas covered: This review focuses on canakinumab, a fully human anti IL-1β antibody, treatment in FMF. Expert commentary: Canakinumab became the first approved therapy by the Food and Drug Administration for FMF very recently, which highlights its importance as the alternative treatment in FMF.
家族性地中海热（FMF）是所有遗传性自身炎症综合征中最常见的一种。其特征为发热和浆膜炎反复发作。若不治疗，可能会并发AA型淀粉样变性。它由编码参与白细胞介素-1β调节的吡喃素的MEFV基因突变引起。治疗的主要药物是秋水仙碱，然而，一部分患者由于反应不充分或不耐受而需要替代治疗。越来越多的数据表明，抗白细胞介素-1药物在治疗对秋水仙碱耐药的FMF病例和改善其生活质量方面是有效的。涵盖领域：本综述重点关注卡那单抗，一种全人源抗白细胞介素-1β抗体，在FMF中的治疗。专家评论：卡那单抗最近成为美国食品药品监督管理局批准的首个用于FMF的疗法，这凸显了其作为FMF替代治疗的重要性。
