Systemic sclerosis sine scleroderma is more aggressive in children than in adults
Authors' conclusion
Compared with adults where ssSSc generally has an indolent course, children present with aggressive disease that heralds a worse prognosis characterized by high cardiorespiratory morbidity and mortality.
Study context
- Population
- Patients with SSc sine scleroderma (never skin involvement; RP and/or digital vasculopathy, positive ANA, typical internal organ involvement, no other defined CTD)
- Participants
- 18 vs 38
- Exposure
- Juvenile-onset SSc sine scleroderma vs adult-onset ssSSc
- ssJSScn = 18
- Adult ssSScn = 38reference
- Outcome
- Clinical and laboratory features, ssJSSc vs adult-onset ssSSc
Methodological detailscomparative, juvenile cases from hospital records, literature case reports and PReS JSSc registry, adult Padua cohort
- Comparative
- Juvenile cases from hospital records, literature case reports and PReS JSSc registry
- Adult Padua cohort
Main finding
As reported in the abstract
Worse outcome in ssJSSc: 6 (33%) died (n = 3) or reached end-stage organ failure (n = 3) vs 2 deaths (5.3%) in adult cohort
33% vs 5.3%
ACA significantly lower in children than adults
20.0% vs 68.4%
P = 0.001
Observational data: associations, not effects.
Further findings
Death or end-stage organ failure · outcome significantly worse in ssJSSc
- Death or end-stage organ failure: ssJSSc: 3 died, 3 end-stage organ failure; adults: 2 deaths
Original publication
Systemic sclerosis sine scleroderma is more aggressive in children than in adults. Rheumatology (Oxford). Published 22 May 2024. doi:10.
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