Açarı Ceyhun, Çomak Elif, Çekiç Şükrü, Türkuçar Serkan, Adıgüzel Dündar Hatice, Şebnem Kılıç Sara, Akman Sema, Makay Balahan, Erbil Ünsal Şevket
Department of Pediatrics, Division of Pediatric Rheumatology, Dokuz Eylül University, Faculty of Medicine, Izmir, Turkey.
Department of Pediatrics, Division of Pediatric Nephrology, Akdeniz University, Faculty of Medicine, Antalya, Turkey.
Arch Rheumatol. 2021 Feb 8;36(3):419-426. doi: 10.46497/ArchRheumatol.2021.8137. eCollection 2021 Sep.
This study aims to evaluate demographic, clinical, and radiological characteristics of Turkish children with chronic non-bacterial osteomyelitis.
Between January 2008 and December 2018, a total of 28 patients (10 males, 18 females; median age: 12.5 years; range, 4.5 to 21 years) who were diagnosed with chronic non-bacterial osteomyelitis in three pediatric rheumatology centers were retrospectively analyzed. The demographic, clinical, and laboratory findings of the patients were recorded.
The median age at the time of diagnosis was 10.2 years. The median time from symptom onset to diagnosis was 6.5 months. The median follow-up was 18.5 months. The most frequent symptom at onset was arthralgia (75.0%). The most frequently involved bone was the femur (67.9%). Eight (63.6%) of 12 patients had at least one Mediterranean fever gene (MEFV) mutation, indicating a significantly higher prevalence than the Turkish population (14.8%). Five of these patients fulfilled the diagnostic criteria for familial Mediterranean fever (FMF). All patients received non-steroidal anti-inflammatory drugs. Other drugs were methotrexate (46.4%), sulfasalazine (39%), corticosteroids (25%), anti-tumor necrosis factor (anti-TNF) agents (32%), pamidronate (25%), and colchicine (21.4%). Six of eight patients with MEFV mutations were administered with colchicine, and all of them responded to treatment.
Clinical evolution and imaging investigations should be carefully performed to prevent any delay in the diagnosis of patients with chronic non-bacterial osteomyelitis. Based on our study results, FMF coexistence is worth investigating in patients with chronic non-bacterial osteomyelitis, particularly in the Turkish population.
本研究旨在评估患有慢性非细菌性骨髓炎的土耳其儿童的人口统计学、临床和放射学特征。
回顾性分析2008年1月至2018年12月期间在三个儿科风湿病中心被诊断为慢性非细菌性骨髓炎的28例患者（10例男性，18例女性；中位年龄：12.5岁；范围4.5至21岁）。记录患者的人口统计学、临床和实验室检查结果。
诊断时的中位年龄为10.2岁。从症状出现到诊断的中位时间为6.5个月。中位随访时间为18.5个月。发病时最常见的症状是关节痛（75.0%）。最常受累的骨骼是股骨（67.9%）。12例患者中有8例（63.6%）至少有一个地中海热基因（MEFV）突变，表明其患病率显著高于土耳其人群（14.8%）。其中5例患者符合家族性地中海热（FMF）的诊断标准。所有患者均接受非甾体类抗炎药治疗。其他药物包括甲氨蝶呤（46.4%）、柳氮磺胺吡啶（39%）、皮质类固醇（25%）、抗肿瘤坏死因子（抗TNF）药物（32%）、帕米膦酸盐（25%）和秋水仙碱（21.4%）。8例MEFV突变患者中有6例接受了秋水仙碱治疗，且所有患者治疗均有反应。
应仔细进行临床评估和影像学检查，以防止慢性非细菌性骨髓炎患者的诊断出现任何延误。根据我们的研究结果，慢性非细菌性骨髓炎患者，尤其是土耳其人群，值得调查是否并存FMF。
