Recovery from deafness in a patient with Muckle-Wells syndrome treated with anakinra.
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.
Mirault
Tristan
T
Claude-Huriez Hospital, Department of Internal Medicine, National Center for Vascular Manifestations of Scleroderma, Lille 2 University, Lille, France.
Launay
David
D
Cuisset
Laurence
L
Hachulla
Eric
E
Lambert
Marc
M
Queyrel
Viviane
V
Quemeneur
Thomas
T
Morell-Dubois
Sandrine
S
Hatron
Pierre-Yves
PY
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*
D018501:Antirheumatic Agents; C507763:IL1RN protein, human; D053590:Interleukin 1 Receptor Antagonist Protein; D012795:Sialoglycoproteins