Kişla Ekinci Rabia Miray, Balci Sibel, Ufuk Altintaş Derya, Yilmaz Mustafa
Department of Pediatric Rheumatology, Çukurova University Faculty of Medicine, Adana, Turkey.
Department of Pediatric Allergy and Immunology, Çukurova University Faculty of Medicine, Adana, Turkey.
Arch Rheumatol. 2017 Oct 30;33(3):282-287. doi: 10.5606/ArchRheumatol.2018.6488. eCollection 2018 Sep.
This study aims to describe the effects of concomitant disorders on the course of familial Mediterranean fever (FMF) and the relevance of genotype on these associations.
Files of 494 FMF patients (257 males, 237 females; mean age 12.8±1.94 years; range 1.6 to 23 years) were retrospectively examined. Age of diagnosis, sex, MEditerrenean FeVer mutations, colchicine dosage, disease severity score and concomitant diseases in FMF course were recorded. FMF diagnoses were based on Tel-Hashomer criteria and disease severity was determined by international severity scoring system for FMF. Patients were divided into two groups as M694V positives and M694V negatives. We compared the groups in terms of accompanying illnesses, MEditerrenean FeVer mutations, and disease severity scores among five concomitant diseases: juvenile idiopathic arthritis (JIA), asthma, Henoch- Schonlein purpura, periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome, and others.
The mean age at diagnosis was 8.7±1.9 years. Eighty-five patients (17.2%) had accompanying diseases including JIA, asthma, periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome, and Henoch-Schonlein purpura. Mean disease severity scores were 2.4±1.1 in patients with only FMF and 3.0±1.5 in patients with concomitant disorders (p=0.001). Patients with concomitant JIA showed the highest severity scores (4.3±1.6). A statistically significant difference was found with one-way analysis of variance.
Our findings indicate that concomitant diseases, particularly JIA, influence FMF severity. Therefore, it may be beneficial to focus on diagnosis and treatment of comorbid inflammatory diseases, which may worsen the course of FMF.
本研究旨在描述并发疾病对家族性地中海热（FMF）病程的影响以及基因型与这些关联的相关性。
对494例FMF患者（男性257例，女性237例；平均年龄12.8±1.94岁；范围1.6至23岁）的病历进行回顾性检查。记录诊断年龄、性别、地中海热突变、秋水仙碱剂量、疾病严重程度评分以及FMF病程中的并发疾病。FMF诊断基于Tel-Hashomer标准，疾病严重程度由FMF国际严重程度评分系统确定。患者分为M694V阳性和M694V阴性两组。我们比较了两组在五种并发疾病（幼年特发性关节炎（JIA）、哮喘、过敏性紫癜、周期性发热、口疮性口炎、咽炎和腺炎综合征）及其他疾病方面的伴随疾病、地中海热突变和疾病严重程度评分。
诊断时的平均年龄为8.7±1.9岁。85例患者（17.2%）患有包括JIA、哮喘、周期性发热、口疮性口炎、咽炎和腺炎综合征以及过敏性紫癜在内的伴随疾病。仅患有FMF的患者平均疾病严重程度评分为2.4±1.1，并发疾病患者为3.0±1.5（p = 0.001）。并发JIA的患者严重程度评分最高（4.3±1.6）。单因素方差分析发现有统计学显著差异。
我们的研究结果表明，并发疾病，尤其是JIA，会影响FMF的严重程度。因此，关注可能使FMF病程恶化的合并炎症性疾病的诊断和治疗可能有益。
