Sjögren's disease & overlap diseases

Clinical characteristics of primary SS– and overlap SS–associated pulmonary arterial hypertension: a multicentre retrospective study

Rheumatology (Oxford)Published Retrospective cohort study

Authors' conclusion

Overlap SS-PAH generally has a better prognosis than pSS-PAH, with improved exercise capacity and milder haemodynamic abnormalities. However, overlap with PBC/AIH is associated with a poorer prognosis.

Study context

Population
Patients with SS-associated PAH diagnosed by right heart catheterization (RHC), March 2013–March 2024, four Chinese medical centres
Exposure
Overlap SS-PAH vs pSS-PAH
Outcome
Event-free survival
Methodological detailsmulticentre, four Chinese medical centres
  • Multicentre
  • Four Chinese medical centres

Main finding

As reported in the abstract

  • Better event-free survival in overlap SS-PAH vs pSS-PAH

Observational data: associations, not effects.

Further findings

Clinical and haemodynamic characteristics

Overlap SS-PAH: lower proportion in WHO functional class III-IV, lower pulmonary vascular resistance (PVR), higher cardiac index vs pSS-PAH
Overlap SS-PAH: higher treatment success rates
Overlap SS-PAH most commonly associated with SLE

Overlap SS-PAH with PBC or autoimmune hepatitis (AIH) (subgroup) · 1 year

Significantly lower 1-year event-free survival, older age and elevated ALP levels

Original publication

Clinical characteristics of primary SS– and overlap SS–associated pulmonary arterial hypertension: a multicentre retrospective study. Rheumatology (Oxford). Published 10 January 2025. doi:10.1093/rheumatology/keaf013

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

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