Sjögren's disease & overlap diseases
Clinical characteristics of primary SS– and overlap SS–associated pulmonary arterial hypertension: a multicentre retrospective 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 →