Systemic sclerosis & Raynaud's
Treatment strategies and survival of patients with connective tissue disease and pulmonary arterial hypertension: a COMPERA analysis
Authors' conclusion
Mortality remains high in patients with CTD-PAH, especially for patients with SSc-PAH.
Study context
- Population
- Patients with newly diagnosed pulmonary arterial hypertension (PAH) associated with CTD in COMPERA, a European PH registry
- Participants
- 607 patients
- Exposure
- Survival by underlying CTD (SSc, SLE, MCTD, UCTD, other CTD); in SSc-PAH, initial ERA-PDE5i combination vs ERA or PDE5i monotherapy
- SSc-PAHn = 390
- SLE-PAHn = 34
- MCTD-PAHn = 33
- UCTD-PAHn = 60
- Other CTD-PAHn = 90
- Outcome
- All-cause mortality according to the underlying CTD
Methodological detailsEuropean pulmonary hypertension registry (COMPERA), multivariable adjustment
- European pulmonary hypertension registry (COMPERA)
- Multivariable adjustment
Main finding
As reported in the abstract
Survival estimates at 1, 3 and 5 years
SSc-PAH
85%, 59% and 42%
SLE-PAH
97%, 77% and 61%
MCTD-PAH
97%, 70% and 59%
UCTD-PAH
88%, 67% and 52%
Other CTD-PAH
92%, 69% and 55%
P = 0.001
Observational data: associations, not effects.
Further findings
| Outcome | P value |
|---|---|
| Survival in SSc-PAH by initial therapyInitial ERA-PDE5i combination vs Initial ERA monotherapy · survival significantly better with combination | P = 0.016 |
| Survival in SSc-PAH by initial therapyInitial ERA-PDE5i combination vs Initial PDE5i monotherapy · survival significantly better with combination | P = 0.012 |
Original publication
Treatment strategies and survival of patients with connective tissue disease and pulmonary arterial hypertension: a COMPERA analysis. Rheumatology (Oxford). Published 18 July 2023. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →