Efficacy and Safety of Anakinra in Colchicine-Resistant or -Intolerant Familial Mediterranean Fever: A Single-Center Real-Life Experience

阿那白滞素治疗对秋水仙碱耐药或不耐受的家族性地中海热的疗效和安全性:单中心真实世界经验

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Abstract

Familial Mediterranean Fever (FMF) is characterized by recurrent febrile attacks and serositis. While colchicine is the primary treatment for FMF, some patients present resistance or intolerance with respect to this drug. Anakinra-an IL-1 receptor antagonist-has demonstrated efficacy in colchicine-resistant or -intolerant FMF patients. Background and Objectives: This study aimed to evaluate the clinical characteristics, treatment duration, response to therapy, dose interval modifications, and long-term outcomes in FMF patients treated with anakinra. Materials and Methods: We retrospectively analyzed data from 68 FMF patients who were colchicine-resistant or -intolerant and received anakinra treatment. Results: The median patient age was 40.2 years, with a predominance of female patients (57.3%). The median follow-up duration for patients treated with anakinra was 34.2 months. Anakinra dosing was successfully extended in 30.8% of patients. Eight patients discontinued anakinra due to remission, with a median remission duration of 18.4 months. In a subgroup analysis of 57 patients treated with anakinra for at least 12 months, a significant decrease was observed in Pras scores at 0 months, 3 months, and 12 months, as well as in Erythrocyte Sedimentation Rate, C-reactive protein, and Serum Amyloid A values (all p < 0.001). Statistically significant decreases in 24 h proteinuria values were found between 0 and 3 months, 3 and 12 months, and 0 and 12 months (p = 0.011, p = 0.006, and p = 0.007, respectively). Anakinra use in pregnancy and kidney transplant recipients was well tolerated. Dose extension and treatment discontinuation in remission are feasible strategies. Conclusions: These findings support the use of anakinra as a good treatment option in selected patients.

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