Titre :

Socio-professional impact and quality of life of cryopyrin-associated periodic syndromes in 54 patients in adulthood.

Auteur(s) :

Vasse, M. [Auteur]
Centre Hospitalier Régional Universitaire [CHU Lille] [CHRU Lille]
Reumaux, H. [Auteur]
Centre Hospitalier Régional Universitaire [CHU Lille] [CHRU Lille]
Koné-Paut, I. [Auteur]
Centre de Référence des Maladies Auto-Inflammatoires et des Amyloses [CH Versailles] [CeRéMAIA - Hôpital André Mignot]
Quartier, P. [Auteur]
Hôpital Necker - Enfants Malades [AP-HP]
Hachulla, Eric [Auteur]
Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286

Titre de la revue :

Clinical and experimental rheumatology

Nom court de la revue :

Clin Exp Rheumatol

Date de publication :

2023-03-14

ISSN :

0392-856X

Discipline(s) HAL :

Sciences du Vivant [q-bio]

Résumé en anglais : [en]

OBJECTIVES: Cryopyrin-associated periodic syndromes (CAPS) belongs to the group of hereditary recurrent fever disorders characterised by interleukin1β-mediated systemic inflammation. Specific treatment by IL-1 targeting ...
Lire la suite >OBJECTIVES: Cryopyrin-associated periodic syndromes (CAPS) belongs to the group of hereditary recurrent fever disorders characterised by interleukin1β-mediated systemic inflammation. Specific treatment by IL-1 targeting drugs has significantly modified the disease evolution. We aimed to evaluate the socio-professional impact of CAPS in the long term and the influence of genetic variants in the phenotype. METHODS: We made a multicentre, observational and descriptive study and collected retrospective data from childhood to adulthood, and until the last year of follow-up. We assessed the quality of life (QoL) of the patients by phone interviews. We also used the SF36 questionnaire including 8 domains: physical function, physical role, body pain, general health, vitality, social function, emotional role and mental health. A high score means a better QoL. RESULTS: Fifty-four patients were evaluated (14 familial cold autoinflammatory syndrome, 27 Muckle-Wells syndrome, 7 chronic infantileneurological cutaneous and articular syndrome. The study showed improvement in symptoms in adulthood and good QoL in all domains apart from school (87%) and work (61%) absenteeism. The MWS group is intermediate in terms of symptoms but seems to describe a better QoL compared to the other groups. The genetic variant alone does not determine the expression of the disease. CONCLUSIONS: Our study shows that CAPS patients have an improvement of symptoms in adulthood and a satisfactory QoL for most of them. Anti-IL1 treatment is the main factor linked to this improvement and therefore early initiation should be encouraged.Lire moins >

Langue :

Anglais

Audience :

Internationale

Vulgarisation :

Non

Établissement(s) :

Université de Lille
Inserm
CHU Lille

Collections :

• Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286

Date de dépôt :

2024-01-11T23:11:39Z
2024-03-04T11:09:58Z