Autoinflammatory & rare systemic diseases
Socio-professional impact and quality of life of cryopyrin-associated periodic syndromes in 54 patients in adulthood
Authors' conclusion
Our study shows that CAPS patients have an improvement of symptoms in adulthood and a satisfactory QoL for most of them.
Study context
- Population
- Adults with cryopyrin-associated periodic syndromes (CAPS), followed from childhood to adulthood
- Participants
- 54 adults
- Exposure
- Familial cold autoinflammatory syndrome vs MWS vs chronic infantileneurological cutaneous and articular syndrome
- Familial cold autoinflammatory syndromen = 14
- MWSn = 27
- Chronic infantileneurological cutaneous and articular syndromen = 7
- Outcome
- Socio-professional impact of CAPS in the long term and QoL (SF36, 8 domains; higher score = better QoL)
Methodological detailsretrospective data, phone interviews, SF36 questionnaire
- Retrospective data
- Phone interviews
- SF36 questionnaire
Main finding
As reported in the abstract
School absenteeism
87%
Work absenteeism
61%
Observational data: associations, not effects.
Further findings
Influence of genetic variants on phenotype
- The genetic variant alone does not determine the expression of the disease
Original publication
Socio-professional impact and quality of life of cryopyrin-associated periodic syndromes in 54 patients in adulthood. Clin Exp Rheumatol. Published 5 October 2022. doi:10.
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