Socio-professional impact and quality of life of cryopyrin-associated periodic syndromes in 54 patients in adulthood. - Université de Lille Accéder directement au contenu
Article Dans Une Revue Clinical and experimental rheumatology Année : 2023

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

Résumé

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.

Dates et versions

hal-04488099 , version 1 (04-03-2024)

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M. Vasse, H. Reumaux, I. Koné-Paut, P. Quartier, Eric Hachulla. Socio-professional impact and quality of life of cryopyrin-associated periodic syndromes in 54 patients in adulthood.. Clinical and experimental rheumatology, 2023, Clinical and experimental rheumatology, ⟨10.55563/clinexprheumatol/bfyb6o⟩. ⟨hal-04488099⟩

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