Systemic sclerosis & Raynaud's
Juvenile and adult-onset scleroderma: Different clinical phenotypes
Authors' conclusion
Diffuse disease subset is the dominant form among juvenile patients, whereas limited form is the main disease subset among adults. On the other hand, juvenile-onset patients have a better survival than those with adult-onset.
Study context
- Population
- Pediatric and adult scleroderma patients
- Exposure
- Juvenile-onset vs adult-onset systemic sclerosis (SSc)
- Outcome
- Clinical features, treatment modalities and survival rates
Methodological detailsKaplan-Meier survival analysis, multiple regression analysis
- Kaplan-Meier survival analysis
- Multiple regression analysis
Main finding
As reported in the abstract
Clinical features and survival
- Cutaneous subset: aSSc mostly limited cutaneous (74.1%), jSSc mostly diffuse cutaneous (76.7%), p<0.001
- Mortality rate significantly higher among adults (p = 0.005)
Observational data: associations, not effects.
Further findings
ILD frequency
P < 0.001
Age at disease onset
Age at diagnosis
Follow-up duration
| Outcome | aSSc | jSSc | P value |
|---|---|---|---|
| Systemic hypertension frequency | 17.9% | 0 events | P = 0.009 |
Independent risk factors of mortality (both aSSc and jSSc)
- Interstitial lung disease (ILD)
- P = 0.03
- Cardiac insufficiency
- P = 0.05
Parts of this Brief are withheld: the automatic checks could not confirm them against the abstract.
Original publication
Juvenile and adult-onset scleroderma: Different clinical phenotypes. Semin Arthritis Rheum. Published 29 March 2023. doi:10. 1016/ j. semarthrit. 2023. 152197
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