Sjögren's disease & overlap diseases

Childhood-onset versus adult-onset Sjögren Syndrome: Clinical phenotype, disease activity, and predictors of organ damage in a national multicenter cohort

Semin Arthritis RheumPublished Retrospective cohort study

Authors' conclusion

coSS displays a distinct clinical phenotype and higher initial disease activity than aoSS, yet accrues less damage over time.

Study context

Population
Patients with Sjögren syndrome, national multicenter cohort
Participants
197 patients
Exposure
Childhood-onset vs adult-onset Sjögren syndrome
  • coSSn = 115
  • Adult-onset Sjögren syndrome (aoSS)n = 82reference
Outcome
Damage (SSDDI ≥1) and its independent predictors
Methodological detailsnational, multicenter, logistic regression
  • National
  • Multicenter
  • Logistic regression

Main finding

As reported in the abstract

Damage (SSDDI ≥1): independent predictors

Older age at symptom onset, OR

1.044

p<0.001

Higher baseline ESSDAI, OR

1.121

p=0.002

Leukopenia, OR

2.657

p=0.046

RF positivity (protective), OR

0.343

p=0.008

Observational data: associations, not effects.

Further findings

Female sex

P = 0.04

Fulfilment of 2016 ACR/EULAR criteria at baseline

P < 0.001

Diagnostic delay

P = 0.001

OutcomecoSSaoSSP value
Baseline ESSDAI9.35.0P < 0.001
SSDDI at last visit · lower in coSS
P = 0.002

Clinical phenotype

coSS: more symptomatic parotitis, cervical lymphadenopathy, constitutional symptoms and RP
aoSS: more sicca symptoms, anti-Ro and RF positivity, leukopenia, lymphopenia and pulmonary involvement

Original publication

Childhood-onset versus adult-onset Sjögren Syndrome: Clinical phenotype, disease activity, and predictors of organ damage in a national multicenter cohort. Semin Arthritis Rheum. Published 18 August 2026. doi:10.1016/j.semarthrit.2026.153060

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