Department of Pediatric Rheumatology, University of Health Sciences, Kanuni Sultan Süleyman Research and Training Hospital, Istanbul, Turkey.
Department of Pediatric Rheumatology, University of Health Sciences, Başakşehir Çam ve Sakura City Hospital, Istanbul, Turkey.
Adv Rheumatol. 2021 Jun 5;61(1):29. doi: 10.1186/s42358-021-00188-2.
Familial Mediterranean fever (FMF) is an autoinflammatory disease characterized by recurrent episodes of fever and serositis. Sacroiliitis can be observed in some FMF patients. This study aimed to compare the demographic, clinical, and laboratory findings, and treatment in children with FMF and sacroiliitis, and children with juvenile spondyloarthropathy (JSpA).
In total, 1687 pediatric FMF patients that were followed-up between May 2010 and June 2020 were evaluated retrospectively. Among them, those with sacroiliitis (n = 63) were included in the study and compared to patients with JSpA (n = 102).
The study included 63 FMF patients with sacroiliitis (38 males [60.3%] and 25 females [39.7%]) with a mean age of 15.2 ± 4.1 years. Mean age at symptom onset was 7.2 ± 5.05 years and mean age at diagnosis was 9.74 ± 4.67 years. The most common mutation in the FMF patients was M694V/M694V (n = 22). Patients were diagnosed with sacroiliitis with a mean of 12 months (range: 6-36 months) after the diagnosis of FMF. Among the FMF patients, 28 (44.4%) had enthesitis, 23 (36.5%) had heel pain, and 11 (17.4%) had low back pain. The study also included 102 JSpA patients (90 males [88.2%] and 12 females [11.8%]). Mean age of patients with JSpA was 16.1 ± 2.8 years. As compared to 102 JSpA patients, patients with FMF and sacroiliitis had higher acute phase reactants, whereas HLA-B27 positivity rate was lower. In addition, axial involvement rate was higher in the JSpA patients.
Sacroiliitis is a common co-morbidity in FMF patients. The phenotypic features of these patients are different from patients with JSpA.
家族性地中海热（FMF）是一种以反复发作的发热和浆膜炎为特征的自身炎症性疾病。一些 FMF 患者可观察到骶髂关节炎。本研究旨在比较 FMF 伴骶髂关节炎和幼年特发性脊柱关节炎（JSpA）患儿的人口统计学、临床和实验室检查结果及治疗情况。
回顾性分析 2010 年 5 月至 2020 年 6 月期间随访的 1687 例儿科 FMF 患者。其中，有骶髂关节炎（n=63）的患儿被纳入研究，并与 JSpA 患儿（n=102）进行比较。
本研究纳入 63 例 FMF 伴骶髂关节炎患儿（38 名男性[60.3%]和 25 名女性[39.7%]），平均年龄为 15.2±4.1 岁。症状起始年龄平均为 7.2±5.05 岁，诊断年龄平均为 9.74±4.67 岁。FMF 患者最常见的突变是 M694V/M694V（n=22）。FMF 患者诊断为骶髂关节炎的平均时间为 FMF 诊断后 12 个月（范围：6-36 个月）。在 FMF 患者中，28 例（44.4%）有肌腱附着点炎，23 例（36.5%）有足跟痛，11 例（17.4%）有腰痛。本研究还纳入了 102 例 JSpA 患者（90 名男性[88.2%]和 12 名女性[11.8%]）。JSpA 患者的平均年龄为 16.1±2.8 岁。与 102 例 JSpA 患者相比，FMF 伴骶髂关节炎患者的急性期反应物更高，而 HLA-B27 阳性率较低。此外，JSpA 患者的轴性受累率更高。
骶髂关节炎是 FMF 患者常见的共患病。这些患者的表型特征与 JSpA 患者不同。
