Systemic sclerosis & Raynaud's
Pulmonary Arterial Hypertension Incidence in Patients With Systemic Sclerosis Treated With Bosentan for Digital Ulcers: Evidence From the SPRING-SIR Registry
Authors' conclusion
In our cohort, the presence of DU was confirmed as a leading risk factor for PAH development, and BOS use for DU prevention may reduce this risk.
Study context
- Population
- Patients with systemic sclerosis (SSc) (2013 ACR/EULAR criteria) from the Sclerosis Progression Investigation (SPRING) registry, no known pulmonary arterial hypertension (PAH) at baseline, ≥1 year of follow-up between 2015 and 2020median follow-up 2.0 years
- Participants
- 727 patients
- Exposure
- Digital ulcers (DU) patients exposed to bosentan vs DU patients never exposed to bosentan vs patients without DU never exposed to bosentan
- Outcome
- Incidence of PAH diagnosed with right heart catheterization
Methodological detailsSPRING registry, Kaplan-Meier analysis, multivariate regression
- SPRING registry
- Kaplan-Meier analysis
- Multivariate regression
Main finding
As reported in the abstract
PAH incidence
DU, never bosentan (BOS)
4.90
Incidence rate (IR) · per 100 PYs
- PAH developed in overall cohort: 54 (7.4%)
- IR 3.71 per 100 PYs
Observational data: associations, not effects.
Further findings
Risk factors independently associated with PAH development
HR
- DU
- 1.86
- Age
- 1.05
- Modified Rodnan skin score > 4
- 2.07
- ILD
- 2.29
- Acetylsalicylic acid treatment
- 1.78
- Multivariate regression
Original publication
Pulmonary Arterial Hypertension Incidence in Patients With Systemic Sclerosis Treated With Bosentan for Digital Ulcers: Evidence From the SPRING-SIR Registry. J Rheumatol. Published 15 January 2025. doi:10.
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