Early onset is an indication of the severity of DADA2 disease
Authors' conclusion
Early-onset DADA2 may be more severe. Early administration of TNF inhibitor can effectively reduce recurrence and quickly alleviate the disease.
Study context
- Population
- DADA2 patients from six families; literature review of paediatric DADA2plus 51 paediatric patients in 4 articles
- Participants
- 6 patients
- Exposure
- Early-onset vs late-onset DADA2
Methodological detailsgenetic analysis (Sanger sequencing of ADA2), literature review of paediatric DADA2
- Genetic analysis (Sanger sequencing of ADA2)
- Literature review of paediatric DADA2
Main finding
As reported in the abstract
- More organs involved in early-onset than late-onset DADA2; high inflammatory responses (elevated ESR, SF, serum amyloid A, CRP)
- Disease severity not significantly different between missense and frameshift mutation
- Early TNFi might result in better remission and reduce recurrence
- Literature (51 paediatric patients): fever, stroke, peripheral nervous system involvement, hypogammaglobulinaemia and hypertension more frequent in early onset
Observational data: associations, not effects.
Original publication
Early onset is an indication of the severity of DADA2 disease. Rheumatology (Oxford). Published 26 April 2022. doi:10.
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