title: Recovery from deafness in a patient with Muckle-Wells syndrome treated with anakinra.
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.
authors.affiliation: Claude-Huriez Hospital, Department of Internal Medicine, National Center for Vascular Manifestations of Scleroderma, Lille 2 University, Lille, France.
authors.forename: Tristan
authors.initials: T
authors.lastname: Mirault
authors.forename: David
authors.initials: D
authors.lastname: Launay
authors.forename: Laurence
authors.initials: L
authors.lastname: Cuisset
authors.forename: Eric
authors.initials: E
authors.lastname: Hachulla
authors.forename: Marc
authors.initials: M
authors.lastname: Lambert
authors.forename: Viviane
authors.initials: V
authors.lastname: Queyrel
authors.forename: Thomas
authors.initials: T
authors.lastname: Quemeneur
authors.forename: Sandrine
authors.initials: S
authors.lastname: Morell-Dubois
authors.forename: Pierre-Yves
authors.initials: PY
authors.lastname: Hatron
mesh_terms: D000328:Adult; D000686:Amyloidosis / Q000188:drug therapy*; D018501:Antirheumatic Agents / Q000627:therapeutic use*; D003638:Deafness / Q000188:drug therapy*; D005260:Female; D006801:Humans; D053590:Interleukin 1 Receptor Antagonist Protein; D020127:Recovery of Function; D012795:Sialoglycoproteins / Q000627:therapeutic use*; D013577:Syndrome; D014581:Urticaria / Q000188:drug therapy*
chemical_list: D018501:Antirheumatic Agents; C507763:IL1RN protein, human; D053590:Interleukin 1 Receptor Antagonist Protein; D012795:Sialoglycoproteins