Skip to main content

Juvenile and Adult-onset Scleroderma Differ

May 15, 2023 5:09 pm

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.


If you are a health practitioner, you may to comment.

Due to the nature of these comment forums, only health practitioners are allowed to comment at this time.

The author has no conflicts of interest to disclose related to this subject