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Identification of factors for persistence of AA amyloidosis in patients with Familial Mediterranean Fever

  • Alexandre Terré
    Affiliations
    Department of Internal Medicine, Centre de Référence des Maladies Auto-Inflammatoires et des Amyloses d'origine Inflammatoire (CEREMAIA), Sorbonne University, AP-HP, Tenon Hospital, 20, Rue de la Chine GRC-28, Paris 75020, France

    Institut Imagine, INSERM U1163, CNRS ERL 8254, Laboratoire d'Excellence GR-Ex, Université Paris Descartes, Sorbonne Paris-Cité, Paris, France
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  • Léa Savey
    Affiliations
    Department of Internal Medicine, Centre de Référence des Maladies Auto-Inflammatoires et des Amyloses d'origine Inflammatoire (CEREMAIA), Sorbonne University, AP-HP, Tenon Hospital, 20, Rue de la Chine GRC-28, Paris 75020, France
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  • David Buob
    Affiliations
    Department of Pathology, Sorbonne University, AP-HP, Tenon Hospital, Paris, France
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  • Gilles Grateau
    Affiliations
    Department of Internal Medicine, Centre de Référence des Maladies Auto-Inflammatoires et des Amyloses d'origine Inflammatoire (CEREMAIA), Sorbonne University, AP-HP, Tenon Hospital, 20, Rue de la Chine GRC-28, Paris 75020, France
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  • Sophie Georgin-Lavialle
    Correspondence
    Corresponding author.
    Affiliations
    Department of Internal Medicine, Centre de Référence des Maladies Auto-Inflammatoires et des Amyloses d'origine Inflammatoire (CEREMAIA), Sorbonne University, AP-HP, Tenon Hospital, 20, Rue de la Chine GRC-28, Paris 75020, France
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  • on behalf of theAA Amyloidosis Study Group
    Author Footnotes
    1 Present address: Sorbonne University Assistance Publique-Hôpitaux de Paris., Centre de référence des maladies auto-inflammatoires et des amyloses inflammatoires (CEREMAIA).Members of the AA Amyloidosis Study Group are listed in the Acknowledgements.
  • Author Footnotes
    1 Present address: Sorbonne University Assistance Publique-Hôpitaux de Paris., Centre de référence des maladies auto-inflammatoires et des amyloses inflammatoires (CEREMAIA).Members of the AA Amyloidosis Study Group are listed in the Acknowledgements.
Published:October 10, 2022DOI:https://doi.org/10.1016/j.ejim.2022.09.028
      Familial Mediterranean Fever (FMF) is the most common monogenic auto-inflammatory disease [
      • Georgin-Lavialle S.
      • Ducharme-Benard S.
      • Sarrabay G.
      • Savey L.
      • Grateau G.
      • Hentgen V.
      Systemic autoinflammatory diseases: clinical state of the art.
      ]. Patients display attacks associating recurrent fever, arthritis, serositis and skin manifestations [
      • Georgin-Lavialle S.
      • Ducharme-Benard S.
      • Sarrabay G.
      • Savey L.
      • Grateau G.
      • Hentgen V.
      Systemic autoinflammatory diseases: clinical state of the art.
      ]. The discovery of the MEFV gene associated with the disease was made in the 1990s, and the diagnostic criteria are well established [
      • Gattorno M.
      • Hofer M.
      • Federici S.
      • Vanoni F.
      • Bovis F.
      • Aksentijevich I.
      • et al.
      Classification criteria for autoinflammatory recurrent fevers.
      ]. As a result, FMF has been widely studied and its treatment is well codified. The first-line treatment is colchicine [
      • Ozen S.
      • Demirkaya E.
      • Erer B.
      • Livneh A.
      • Ben-Chetrit E.
      • Giancane G.
      • et al.
      EULAR recommendations for the management of Familial Mediterranean Fever.
      ]. Intolerance or resistance to colchicine is observed in 5 to 10% of cases [
      • Ben-Zvi I.
      • Kukuy O.
      • Giat E.
      • Pras E.
      • Feld O.
      • Kivity S.
      • et al.
      Anakinra for colchicine-resistant Familial Mediterranean Fever: a randomized, double-blind, placebo-controlled trial.
      ], and effective alternative treatments have been developed, especially biotherapies targeting IL-1 (anakinra, canakinumab) [
      • Fayand A.
      • Savey L.
      • Ducharme-Bénard S.
      • Grateau G.
      • Georgin-Lavialle S.
      Prescription of interleukin-1 inhibitors in a French adult cohort of Familial Mediterranean Fever.
      ].

      Keywords

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