Familial Mediterranean fever is associated with a wide spectrum of inflammatory disorders: results from a large cohort study

• Published in: Rheumatology international Vol. 40; no. 1; pp. 41 - 48
• Main Authors: Atas, Nuh, Armagan, Berkan, Bodakci, Erdal, Satis, Hasan, Sari, Alper, Bilge, Nazife Sule Yasar, Salman, Reyhan Bilici, Yardımcı, Gozde Kubra, Babaoglu, Hakan, Guler, Aslihan Avanoglu, Karadeniz, Hazan, Kilic, Levent, Ozturk, Mehmet Akif, Goker, Berna, Haznedaroglu, Seminur, Kalyoncu, Umut, Kasifoglu, Timucin, Tufan, Abdurrahman
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Berlin Heidelberg 2020; Springer Nature B.V
• Subjects: Adult; Behcet Syndrome - epidemiology; Cohort analysis; Cohort Studies; Comorbidity; Cryptogenic Organizing Pneumonia - epidemiology; Familial Mediterranean Fever - epidemiology; Familial Mediterranean Fever - genetics; Familial Mediterranean Fever - physiopathology; Humans; Immunoglobulin A - immunology; Inflammatory Bowel Diseases - epidemiology; Inflammatory diseases; Medicine; Medicine & Public Health; Middle Aged; Psoriasis - epidemiology; Purpura, Schoenlein-Henoch - epidemiology; Pyrin - genetics; Rheumatology; Spondylarthropathies - epidemiology; Turkey - epidemiology; Vasculitis - epidemiology; Vasculitis - immunology; Young Adult; Turkey; Familial Mediterranean fever; Inflammation; Interleukin-1 β; Comorbidity; Spondyloarthritis
• ISSN: 0172-8172; 1437-160X
• DOI: 10.1007/s00296-019-04412-7

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.