Systemic sclerosis & Raynaud's
Predictors and prognosis of pulmonary hypertension complicating interstitial lung disease in systemic sclerosis
Authors' conclusion
Despite vasodilator therapy, survival in SSc-PH was found to be poor, with the presence of concurrent ILD being associated with worse survival.
Study context
- Population
- SSc patients in the Australian Scleroderma Cohort Study diagnosed with pulmonary hypertension (PH) via right-heart catheterisation164 developed incident PH
- Participants
- 1,883 patients
- Exposure
- SSc-PH with concurrent interstitial lung disease (ILD) vs group 1 pulmonary arterial hypertension (PAH)
- Outcome
- Incident PH and ILD at PH diagnosis
- Follow-up
- Median 4.3 (1.7-7.9) years
Methodological detailsconsecutive patients, Kaplan-Meier survival, logistic regression
- Consecutive patients
- Kaplan-Meier survival
- Logistic regression
Main finding
As reported in the abstract
median follow-up 4.3 (1.7-7.9) years
- Incident PH among SSc patients
- 164 (8.7%)
- Extensive ILD at PH diagnosis
- 40.3%
Observational data: associations, not effects.
Further findings
Vasodilatory therapy at PH diagnosis
- Extensive ILD and PH receiving upfront vasodilatory therapy
- 87.5%
- Similar frequency of each PH cohort treated, regardless of presence or severity of ILD
- No difference in tolerability or therapy cessation vs group 1 PAH
Survival with vasodilator therapy in extensive ILD
- Not associated with survival advantage; associated with amelioration of symptoms and improved physical function and quality of life (QoL)
Original publication
Predictors and prognosis of pulmonary hypertension complicating interstitial lung disease in systemic sclerosis. Rheumatology (Oxford). Published 12 March 2024. doi:10.
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