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Hip involvement in familial Mediterranean fever

  • François Rodrigues
    Affiliations
    Service de Médecine Interne, Hôpital Tenon, Sorbonne Université, Assistance Publique-Hôpitaux de Paris, France
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  • Jérémie Sellam
    Affiliations
    Service de Rhumatologie, Hôpital Saint-Antoine, Sorbonne Université, Assistance Publique-Hôpitaux de Paris, Inserm URMS_938, France
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  • Pierre Quartier
    Affiliations
    Paediatric Hematology-Immunology and Rheumatology Unit, Hôpital Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Université de Paris, France

    Rheumatological and Auto-Immune Diseases in Children (RAISE) Rare Diseases Reference Centre, France

    Imagine Institute, Inserm U 1163, Université de Paris, France
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  • Stéphanie Ducharme-Bénard
    Affiliations
    Service de Médecine Interne, Hôpital du Sacré-Cœur de Montréal, Montréal, Québec, Canada
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  • Sophie Georgin-Lavialle
    Correspondence
    Corresponding author at: Service de Médecine Interne, Hôpital Tenon, 4 rue de la Chine, Paris 75020, France.
    Affiliations
    Service de Médecine Interne, Hôpital Tenon, Sorbonne Université, Assistance Publique-Hôpitaux de Paris, France

    French National Reference Center of Autoinflammatory Diseases and Inflammatory Amyloidosis, - CEREMAIA, France
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Published:November 24, 2022DOI:https://doi.org/10.1016/j.ejim.2022.11.008
      Familial Mediterranean fever (FMF) is a monogenic autoinflammatory disorder that is linked to homozygous mutations of the MEFV gene [
      French FMF Consortium
      A candidate gene for familial Mediterranean fever.
      ]. The field of FMF is expanding as many patients with FMF phenotype are heterozygous with monoallelic variants. Arthritis is a frequent manifestation of FMF, involving 50 to 75% of patients [
      • Sohar E.
      • Gafni J.
      • Pras M.
      • Heller H.
      Familial Mediterranean fever. A survey of 470 cases and review of the literature.
      ]. The hip is the third most commonly affected joint after the knee and ankle [
      • Sohar E.
      • Gafni J.
      • Pras M.
      • Heller H.
      Familial Mediterranean fever. A survey of 470 cases and review of the literature.
      ]. Aseptic necrosis of the femoral head [
      • Konarski W.
      • Poboży T.
      • Śliwczyński A.
      • Kotela I.
      • Krakowiak J.
      • Hordowicz M.
      • et al.
      Avascular necrosis of femoral head—overview and current state of the art.
      ] caused by disruption of local blood supply has also been reported in FMF patients. Meanwhile, hip inflammation or coxitis can be caused by axial spondyloarthritis, which develops at an increased rate in FMF patients [
      • Kaşifoğlu T.
      • Çalışır C.
      • Cansu D.Ü.
      • Korkmaz C.
      The frequency of sacroiliitis in familial Mediterranean fever and the role of HLA-B27 and MEFV mutations in the development of sacroiliitis.
      ,
      • Akar S.
      • Soysal O.
      • Balci A.
      • Solmaz D.
      • Gerdan V.
      • Onen F.
      • et al.
      High prevalence of spondyloarthritis and ankylosing spondylitis among familial Mediterranean fever patients and their first-degree relatives: further evidence for the connection.
      ]. Therefore, the literature is controversial as to whether coxitis in FMF is a distinct entity or a manifestation of an associated axial spondyloarthritis. Our objectives were to describe the clinical, functional and radiological features of hip involvement in FMF.
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