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首页> 外文期刊>Advances in Rheumatology >Assessment of effectiveness of anakinra and canakinumab in patients with colchicine-resistant/unresponsive familial Mediterranean fever
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Assessment of effectiveness of anakinra and canakinumab in patients with colchicine-resistant/unresponsive familial Mediterranean fever

机译：评估血清曲线抗性/无反应家族性地中海发热患者Anakinra和Canakinumab的有效性

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Familial Mediterranean fever (FMF) is a hereditary auto-inflammatory disease characterized by recurrent fever and serosal inflammation. Anti-interleukin-1 (Anti-IL-1) treatments are recommended in colchicine resistant and/or intolerant FMF patients. This study aims to evaluate the efficacy of anakinra and canakinumab in FMF patients that are resistant/intolareted to colchicine or complicated with amyloidosis. Between January 2014 and March 2019, 65 patients following-up at Sivas Cumhuriyet University (Medical Faculty Rheumatology-Internal Medicine Department) who were diagnosed with FMF according to the criteria of Tel-Hashomer were included in the study. The laboratory values and clinical features of patients and disease activities were recorded at least every 3?months, and these data were analyzed. Forty-one (63.1%) patients used anakinra (100?mg/day) and 24 (36.9%) patients used canakinumab (150?mg/8?week). The median duration of anti-IL-1 agents use was 7?months (range, 3–30). Fifteen (23.1%) cases were complicated with amyloidosis. Seven (10.8%) patients had renal transplantation. Overall, the FMF 50 score response was 96.9%. In the group that had a glomerular filtration rate (GFR)?≥?60?ml/min/m2, the median proteinuria decreased from 2390?mg/day (range, 1400–7200) to 890?mg/day (range, 120–2750) (p?=?0.008). No serious infections were detected, except in one patient. Anti-IL-1 agents are effective and safe in the treatment of FMF patients. These agents are particularly effective at reducing proteinuria in patients with GFR?≥?60?ml/min/m2, but less effective in cases with FMF associated with arthritis and sacroiliitis. Large and long follow-up studies are now needed to establish the long-term effects of these treatments.
机译：家族地中海发热（FMF）是一种遗传性的自动炎症疾病，其特征是复发性发烧和浆液炎症。抗白细胞介素-1（抗IL-1）治疗，建议在血清曲线和/或不耐受性FMF患者中。本研究旨在评估胰岛和加甘油蛋白在耐殖民氏菌或混溶性或复杂淀粉样症的患者中的疗效。在2019年1月至2019年3月至2019年3月，65名患者在Sivas Cumhuriyetyet大学（医学院系风湿病学 - 内科医学部门）被诊断出根据Tel-Hashomer标准被诊断为FMF的研究。患者和疾病活动的实验室值和临床特征至少每3个月记录，并分析这些数据。四十一（63.1％）患者使用Anakinra（100？Mg /天）和24例（36.9％）患者使用Canakinumab（150？Mg / 8？周）。抗IL-1代理商使用的中值持续时间为7？月（范围，3-30）。十五（23.1％）病例与淀粉样源性症复杂化。七（10.8％）患者患有肾移植。总的来说，FMF 50分数响应为96.9％。在具有肾小球过滤速率（GFR）的基团中？≥≤60？ml / min / m 2，中值蛋白尿从2390毫克/天（范围，1400-7200）降至890？mg /天（范围，120 -2750）（p？= 0.008）。除了一个患者外，没有检测到严重的感染。抗IL-1试剂在治疗FMF患者的治疗中是有效和安全的。这些试剂在GFR患者中还原蛋白尿中的蛋白尿≥？60？mL / min / m 2特别有效，但在与关节炎和骶髂炎相关的FMF病例中，较低的效果。现在需要大而长期的后续研究来建立这些治疗的长期影响。

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