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

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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.

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Funding

This work was supported by grants from Hacettepe University Rheumatology Association (grant no: HRD2018-001).

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Authors and Affiliations

  1. Division of Rheumatology, Department of Internal Medicine, Gazi University Faculty of Medicine, 06100, Ankara, Turkey

    Nuh Atas, Hasan Satis, Reyhan Bilici Salman, Hakan Babaoglu, Aslihan Avanoglu Guler, Hazan Karadeniz, Mehmet Akif Ozturk, Berna Goker, Seminur Haznedaroglu & Abdurrahman Tufan

  2. Division of Rheumatology, Department of Internal Medicine, Hacettepe University Faculty of Medicine, Ankara, Turkey

    Berkan Armagan, Alper Sari, Gozde Kubra Yardımcı, Levent Kilic & Umut Kalyoncu

  3. Division of Rheumatology, Department of Internal Medicine, Eskisehir Osmangazi University Faculty of Medicine, Eskisehir, Turkey

    Erdal Bodakci, Nazife Sule Yasar Bilge & Timucin Kasifoglu

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  1. Nuh Atas
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Contributions

In accordance with ICMJE criteria, NA and AT designed the study and wrote the initial draft of the manuscript. UK, TK, MAO, BG, and SH contributed to the design of the study, the collection and interpretation of data, and the assistance of the preparation of the manuscript. BA, EB, HS, AS, NSYB, RBS, GKY, HB, AAG, HK, and LK contributed to the data collection and interpretation and revised the manuscript. The literature data were searched and analyzed by all authors. All authors approved the final version to be submitted for publication and agree to be accountable for all aspects of the work.

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Correspondence to Nuh Atas.

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Conflict of interest

Nuh Atas, Berkan Armagan, Erdal Bodakci, Hasan Satis, Alper Sari, Nazife Sule Yasar Bilge, Reyhan Bilici Salman, Gozde Kubra Yardımcı, Hakan Babaoglu, Aslihan Avanoglu Guler, Hazan Karadeniz, Levent Kilic, Mehmet Akif Ozturk, Berna Goker, Seminur Haznedaroglu, Umut Kalyoncu, Timucin Kasifoglu, and Abdurrahman Tufan declare that they have no conflicts of interest related to study.

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This study was approved by Gazi University ethical committee (Approval no. 2017-622), conducted in accordance with the 1975 Helsinki Declaration.

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Informed consent was obtained from all participants included in the study.

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Atas, N., Armagan, B., Bodakci, E. et al. Familial Mediterranean fever is associated with a wide spectrum of inflammatory disorders: results from a large cohort study. Rheumatol Int 40, 41–48 (2020). https://doi.org/10.1007/s00296-019-04412-7

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