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.