Muckle–Wells syndrome: clinical perspectives - Université de Montpellier Accéder directement au contenu
Article Dans Une Revue Open Access Rheumatology: Research and Reviews Année : 2017

Muckle–Wells syndrome: clinical perspectives


Muckle-Wells syndrome (MWS) is a rare autoinflammatory disorder. It is due to NLRP3 gene mutations, responsible for excessive caspase-1 activation and interleukin 1β processing. MWS is the intermediate phenotype of severity of cryopyrin-associated periodic syndrome. Urticarial rash, conjunctivitis, recurrent fever, arthralgia, and fatigue are the main clinical manifestations of MWS. Yet, sensorineural hearing loss and renal amyloidosis can occur after long term evolution. Patients' quality of life has been drastically improved with the advent of IL-1 inhibitors. This review reports recent findings in MWS, particularly genotype/phenotype correlation, and discusses the clinical perspectives of this disease in a time of efficient treatment.
Fichier principal
Vignette du fichier
IRMB 49_P51_Muckle-Wells syndrome_clinical perspectives_oarrr-9-123.pdf (428.66 Ko) Télécharger le fichier
Origine : Fichiers éditeurs autorisés sur une archive ouverte

Dates et versions

hal-01870379 , version 1 (24-03-2021)


Paternité - Pas d'utilisation commerciale



Tu-Anh Tran. Muckle–Wells syndrome: clinical perspectives. Open Access Rheumatology: Research and Reviews, 2017, 9, pp.123 - 129. ⟨10.2147/OARRR.S114447⟩. ⟨hal-01870379⟩
67 Consultations
85 Téléchargements



Gmail Facebook X LinkedIn More