Juvenile and Adult-onset Scleroderma Differ Save
A retrospecitve cohort comparision of juvenile (jSSc) and adult-onset (aSSc) systemic sclerosis (SSc) are both rare but present differently.
A single center examined demographic characteristics, clinical features, autoantibody profiles, treatments, outcomes and survival.
A total of 158 adults (age 37 yrs) and 58 juvenile (mean age 8.8 yrs) Scl patients were identified.
Differences between the two cohorts included:
- Interstitial lung disease (ILD): adults (aSSc) had more ILD (50.9% vs 30%, p<0.001) and systemic hypertension (17.9% vs 0, p = 0.009)
- aSSc presented mostly with limited cutaneous subset (74.1%)
- jSSC: was more likely to present with diffuse cutaneous disease (76.7%), (p<0.001).
- Mortality was significantly higher among adults (p = 0.005).
- For both aSSc and jSSc, ILD (p = 0.03) and cardiac insufficiency (p = 0.05) were independent risk factors of mortality
Diffuse disease subset was more dominant among juvenile patients, whereas limited form is the main disease subset among adults. Yet, juvenile-onset patients have a better survival than those with adult-onset SSc.
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