Department of Pediatric Rheumatology, Erciyes University, Kayseri, Turkey.
Department of Pediatric Rheumatology, Kayseri Acibadem Hospital, Kayseri, Turkey.
Mod Rheumatol. 2022 Jan 5;32(1):177-185. doi: 10.1080/14397595.2021.1908502.
Colchicine is the fundamental treatment of familial Mediterranean fever (FMF). Still, 5-10% of patients are not in remission with colchicine treatment. A consensus could not be established for the definition of colchicine resistance in FMF. This study aimed to determine factors that help to predict colchicine resistance in pediatric FMF patients.
Patients with FMF that age of diagnosis was under 18 years old were included in our study. Fifty colchicine responsive and 33 colchicine-resistant patients were stratified as groups 1 and 2, respectively. Patients' clinical and laboratory findings were evaluated. Logistic regression analysis was used to determine the risk factors of colchicine-resistant FMF. Receiver operating characteristic (ROC) curve analysis was used to identify and compare the predictive performances of colchicine-resistant FMF models.
Homozygous exon 10 MEFV mutations were frequent in group 2 (Group 1: 34 (68%), group 2: 32 (97%), p = .013). Univariate analysis showed that the age of onset of symptoms, age of diagnosis, chronic arthritis, myalgia and diarrhea during attacks, and the number of attacks, high ISSF and Pras score, high C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) values under colchicine treatment were risk factors for colchicine-resistant FMF. With multivariate analysis, the number of attacks (OR 1.418, CI (95%) 1.149-1.750, p = .001) and high ESR values (OR 1.129, CI (95%) 1.059-1.204, p<.001) were detected as independent risk factors for colchicine-resistant FMF.
The predictive factors were determined for pediatric colchicine-resistant FMF in our study. The results will help to early diagnosis and treatment of chronic inflammation in FMF.
秋水仙碱是家族性地中海热（FMF）的基本治疗药物。然而，仍有 5-10%的患者对秋水仙碱治疗无缓解。对于 FMF 中秋水仙碱耐药的定义，尚未达成共识。本研究旨在确定有助于预测儿科 FMF 患者秋水仙碱耐药的因素。
纳入年龄在 18 岁以下且被诊断为 FMF 的患者。将 50 例秋水仙碱反应性和 33 例秋水仙碱耐药患者分别分层为第 1 组和第 2 组。评估患者的临床和实验室检查结果。采用逻辑回归分析确定 FMF 秋水仙碱耐药的危险因素。采用受试者工作特征（ROC）曲线分析确定和比较 FMF 秋水仙碱耐药模型的预测性能。
第 2 组中纯合子外显子 10 MEFV 突变较为常见（第 1 组：34 例（68%），第 2 组：32 例（97%），p=0.013）。单因素分析显示，症状发作年龄、诊断年龄、慢性关节炎、肌痛和腹泻发作时、发作次数、高 ISSF 和 Pras 评分、高 C 反应蛋白（CRP）和红细胞沉降率（ESR）值是 FMF 秋水仙碱耐药的危险因素。多因素分析显示，发作次数（OR 1.418，CI（95%）1.149-1.750，p=0.001）和高 ESR 值（OR 1.129，CI（95%）1.059-1.204，p<.001）是 FMF 秋水仙碱耐药的独立危险因素。
本研究确定了儿科秋水仙碱耐药 FMF 的预测因素。研究结果将有助于 FMF 慢性炎症的早期诊断和治疗。
