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Enthesitis may be one of the signs of severe disease in familial Mediterranean fever

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Abstract

Objective

Familial Mediterranean fever (FMF) is an auto-inflammatory disease that is also characterized with some of the common musculoskeletal features of spondyloarthritis (SpA). Enthesitis is the hallmark of SpA. Recently, it was postulated that exertional leg pain is a possible sign of lower extremity enthesitis associated with FMF severity. In this study, we have evaluated the association between the enthesitis, enthesitis score and disease severity in FMF patients.

Methods

We enrolled 238 FMF patients that fulfilled the modified Tel-Hashomer criteria. We assessed the presence of enthesitis at the Maastricht Ankylosing Spondylitis Enthesitis Score (MASES) defined sites with standard palpation method. Then, FMF patients dichotomised two groups as enthesitis group and controls. Herein, we evaluated the enthesis extensity with MASES. FMF disease severity was determined via the international severity scoring system for FMF (ISSF). Firstly, we have compared demographic properties, disease-related features and ISSF scores of the groups. Then, we have correlated ISSF with MASES in enthesitis group.

Results

We showed that 54 (22.6%) of 238 patients had enthesitis. The demographic features were similar between the groups. The enthesitis group had higher ISSF scores (p < 0.001); higher frequency of fever (p = 0.004), exertional leg pain (p < 0.001), myalgia (p < 0.001) and arthritis (p = 0.01); and more intense, widespread, frequent and longer attacks compared with controls. Moreover, there was a weak correlation between ISSF and MASES in the patients with enthesitis.

Conclusion

Enthesitis may be a sign of more severe FMF phenotype and frequently associated with other musculoskeletal manifestations resemble SpA.

Key points

•More than one-fifth of the patients with FMF would suffer from enthesitis.

•The FMF patients with enthesitis had higher ISSF scores; higher frequency of fever, exertional leg pain, myalgia and arthritis; and more intense, widespread, frequent and longer attacks as compared with controls.

•Enthesitis may be a sign of more severe FMF phenotype and frequently associated with other SpA-like musculoskeletal feature.

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Data availability

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Author information

Authors and Affiliations

  1. Department of Rheumatology, Kartal Dr. Lutfi Kirdar Training and Research Hospital, Kartal, 34680, Istanbul, Turkey

    Nesrin Sen, Omur Volkan, Sibel Yilmaz-Oner & Mehmet Engin Tezcan

  2. Department of Internal Medicine, Kartal Dr. Lutfi Kirdar Training and Research Hospital, Istanbul, Turkey

    Mesut Yilmaz & Ezgi Tukel

  3. Department of Internal Medicine, Section of Rheumatology, Namik Kemal University, School of Medicine, Tekirdag, Turkey

    Ridvan Mercan

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  1. Nesrin Sen
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Contributions

NS: Provide important contributions to conceptual or planning stages of the study or collection/processing, analysis or interpretation of the data. MY: Taking responsibility in patient follow-up, collection of relevant biological materials, data management and reporting, execution of the experiments. RM: Taking responsibility in logical interpretation and conclusion of the results. OM: Planning methodology to reach the conclusions. SYO: Reviewing the article before submission scientifically besides spelling and grammar. ET: Taking responsibility in patient follow-up, collection of relevant biological materials, data management and reporting, execution of the experiments. MET: Main writer, planning methodology to reach the conclusions and reviewing the article before submission scientifically besides spelling and grammar

Corresponding author

Correspondence to Mehmet Engin Tezcan.

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Ethics approval

This study was approved by the Local Research Ethics Committee and carried out in compliance with the Helsinki Declaration.

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All the patients gave written informed consent.

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Statistical analyses were carried out using SPSS Version 17.0 (SPSS Inc., Chicago, IL, USA).

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Sen, N., Yilmaz, M., Mercan, R. et al. Enthesitis may be one of the signs of severe disease in familial Mediterranean fever. Clin Rheumatol 40, 1479–1485 (2021). https://doi.org/10.1007/s10067-020-05392-x

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  • DOI: https://doi.org/10.1007/s10067-020-05392-x

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