Recovery from deafness in a patient with Muckle-Wells syndrome treated with anakinra

Authors

  • Tristan Mirault,

    1. Claude-Huriez Hospital, Department of Internal Medicine, National Center for Vascular Manifestations of Scleroderma, Regional University Hospital, Lille 2 University, Lille, France
    Search for more papers by this author
  • David Launay,

    Corresponding author
    1. Claude-Huriez Hospital, Department of Internal Medicine, National Center for Vascular Manifestations of Scleroderma, Regional University Hospital, Lille 2 University, Lille, France
    • Service de Médecine Interne, Hôpital Claude-Huriez, CHRU Lille, Rue Michel Polonovski, F-59037 Lille Cedex, France
    Search for more papers by this author
  • Laurence Cuisset,

    1. Department of Biochemical Genetics, Pavillon Cassini, Hospital and Institut Cochin, Paris, France
    Search for more papers by this author
  • Eric Hachulla,

    1. Claude-Huriez Hospital, Department of Internal Medicine, National Center for Vascular Manifestations of Scleroderma, Regional University Hospital, Lille 2 University, Lille, France
    Search for more papers by this author
  • Marc Lambert,

    1. Claude-Huriez Hospital, Department of Internal Medicine, National Center for Vascular Manifestations of Scleroderma, Regional University Hospital, Lille 2 University, Lille, France
    Search for more papers by this author
  • Viviane Queyrel,

    1. Claude-Huriez Hospital, Department of Internal Medicine, National Center for Vascular Manifestations of Scleroderma, Regional University Hospital, Lille 2 University, Lille, France
    Search for more papers by this author
  • Thomas Quemeneur,

    1. Claude-Huriez Hospital, Department of Internal Medicine, National Center for Vascular Manifestations of Scleroderma, Regional University Hospital, Lille 2 University, Lille, France
    Search for more papers by this author
  • Sandrine Morell-Dubois,

    1. Claude-Huriez Hospital, Department of Internal Medicine, National Center for Vascular Manifestations of Scleroderma, Regional University Hospital, Lille 2 University, Lille, France
    Search for more papers by this author
  • Pierre-Yves Hatron

    1. Claude-Huriez Hospital, Department of Internal Medicine, National Center for Vascular Manifestations of Scleroderma, Regional University Hospital, Lille 2 University, Lille, France
    Search for more papers by this author

Abstract

Muckle-Wells syndrome (MWS) is a dominantly inherited autoinflammatory disease characterized by rashes, fever, arthralgia, sensorineural deafness, and the possible development of systemic AA amyloidosis. We used anakinra to treat a 22-year-old patient with MWS who had deafness and a high serum level of C-reactive protein (CRP). Following treatment with anakinra, the patient's CRP level normalized, and she recovered from deafness. The fact that this occurrence has never been previously reported strengthens the role of anakinra in MWS but also raises new questions about the physiopathology of such deafness.

Ancillary