Systemic sclerosis & Raynaud's
Clinical characteristics and survival of pulmonary arterial hypertension with or without interstitial lung disease in systemic sclerosis
Authors' conclusion
The prevalence of concurrent PAH-ILD in the ASCS is 7%, with poorer survival in those patients with PAH-ILD compared to ILD or SSc alone. The presence of PAH confers a poorer overall prognosis than even extensive ILD
Study context
- Population
- Participants meeting ACR/EULAR criteria for SSc in the Australian Scleroderma Cohort Study
- Participants
- 1,561 participants
- Exposure
- SSc with pulmonary arterial hypertension (PAH)-only, interstitial lung disease (ILD)-only, concurrent PAH and ILD, or neither
- Outcome
- Clinical phenotype and survival (prognosis)
Methodological detailsAustralian Scleroderma Cohort Study, Kaplan-Meier estimates, Cox-regression modelling, multivariable hazard modelling
- Australian Scleroderma Cohort Study
- Kaplan-Meier estimates
- Cox-regression modelling
- Multivariable hazard modelling
Main finding
As reported in the abstract
Mortality (multivariable hazard modelling)
HR for death (95% CI)P value
PAH with extensive ILDworst prognosis
5.65(3.50–9.12)
P < 0.01PAH-onlyvs SSc-only
4.21(2.89–6.13)
P < 0.01PAH with limited ILD
2.46(1.52–3.99)
P < 0.01- Survival was reduced in the PAH-only and PAH and ILD (PAH-ILD) groups
- P < 0.01
Observational data: associations, not effects.
Further findings
Prevalence of groups
Clinical phenotype of PAH-ILD
- PAH-ILD more frequently male, diffuse skin involvement, higher inflammatory markers, older age of SSc onset and more extensive ILD than cohort overall
- P < 0.001
- People of Asian race more frequently developed PAH-ILD
- P < 0.001
Function and health-related quality of life (HRQoL)
- PAH-ILD or PAH-only had worse WHO functional class and 6-min-walk-distance than ILD-only
- P < 0.001
- HRQoL scores worst in PAH-ILD
- P < 0.001
Original publication
Clinical characteristics and survival of pulmonary arterial hypertension with or without interstitial lung disease in systemic sclerosis. Arthritis Res Ther. Published 12 May 2023. doi:10.
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