Abstract

Familial Mediterranean fever (FMF) is characterized by recurrent short-lived/self-limiting inflammatory attacks. Besides these, a substantial number of patients with FMF present with a variety of other inflammatory diseases; however, this issue has not been systematically studied previously. Hence, we aimed to investigate the frequency of inflammatory comorbid diseases in a large FMF cohort. All patients were recruited from “FMF in Central Anatolia (FiCA) Cohort”, comprising 971 (mean age 35.3 ± 12 years, 61.5% female) adult subjects. All patients fulfilled Tel Hashomer criteria. Demographic data, FMF disease characteristics, MEFV gene mutations, and comorbid inflammatory diseases were meticulously questioned, and laboratory features and genotype data were retrieved from hospital records. There were comorbid inflammatory diseases in 205 (21.1%) patients. The most common inflammatory disease was spondyloarthritis (12.9%). Other remarkable inflammatory disorders were psoriasis, immunoglobulin A vasculitis/Henoch–Schönlein purpura, Behçet’s disease and inflammatory bowel diseases. Cryptogenic organizing pneumonia is a newly defined entity in our cohort which is seemed to be associated with FMF (0.3%). Number of patients with persistent inflammation was higher in those with comorbid diseases (p < 0.001). Our results suggest that FMF is commonly associated with other inflammatory diseases. Therefore, clinicians should be cautious about comorbid inflammatory diseases in FMF patients, particularly in those with persistent inflammation. Identification of pathogenic pathways linking FMF to these diseases warrants further investigations.

中文翻译：

---

家族性地中海热与多种炎症性疾病有关：一项大型队列研究的结果

摘要

家族性地中海热（FMF）的特征是反复发作的短暂性/自限性炎症发作。除此之外，大量的FMF患者还患有多种其他炎症性疾病。但是，以前尚未对此问题进行系统的研究。因此，我们的目的是调查大型FMF人群中炎症性合并症的频率。所有患者均来自“中安纳托利亚中部FMF（FiCA）队列”，包括971名（平均年龄35.3±12岁，女性61.5％）成年受试者。所有患者均符合Tel Hashomer标准。认真询问人口统计学数据，FMF疾病特征，MEFV基因突变和共病性炎症，并从医院记录中检索实验室特征和基因型数据。205例共存炎性疾病（21。1％）患者。最常见的炎性疾病是脊椎关节炎（12.9％）。其他显着的炎症性疾病包括牛皮癣，免疫球蛋白A血管炎/过敏性紫癜，白塞氏病和炎症性肠病。隐源性组织性肺炎是我们队列中一个新定义的实体，似乎与FMF有关（0.3％）。合并症患者中持续发炎的患者数量更高（p  <0.001）。我们的结果表明，FMF通常与其他炎症性疾病有关。因此，临床医生应谨慎对待FMF患者（尤其是持续性炎症患者）的合并炎症性疾病。确定将FMF与这些疾病相关的致病途径值得进一步研究。