Systemic sclerosis & Raynaud's

Juvenile and adult-onset scleroderma: Different clinical phenotypes

Semin Arthritis RheumPublished Retrospective cohort study

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

OutcomeaSScjSScP value
Systemic hypertension frequency17.9%0 eventsP = 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

Composed by AI from the published abstract and checked against it. How Briefs are made →

Get them every week

New studies on this topic in My Week and in your Monday e-mail.

We e-mail you a sign-in link and a code. No password, no spam.