New phenotype and outcomes in connective tissue disease-associated pulmonary arterial hypertension: relevance of age-related comorbidities
Authors' conclusion
Contemporary CTD-PAH patients included in REHAP are older and have a lower comorbidity burden than IPAH patients, while exhibiting similar survival rates up to 5 years. CKD is an independent prognostic factor in both aetiologies. CTD-PAH patients tend to receive a more conservative treatment approach.
Study context
- Population
- Patients with CTD-PAH or Idiopathic PAH (IPAH) in the Spanish Registry of PAH (REHAP), 2014–2023
- Participants
- 757 patients
- Exposure
- CTD-PAH vs contemporary IPAH
- CTD-PAHn = 340
- IPAHn = 417reference
- Outcome
- Survival rates
- Follow-up
- 1, 3 and 5 years
Methodological detailsregistry-based (REHAP), Kaplan-Meier survival estimates, Cox proportional-hazards regression
- Registry-based (REHAP)
- Kaplan-Meier survival estimates
- Cox proportional-hazards regression
Main finding
As reported in the abstract
1-year survival
3-year survival
5-year survival
- CTD-PAH vs IPAH: P = 0.884
Observational data: associations, not effects.
Further findings
Age
P < 0.001
Patients with ≥3 comorbidities
P < 0.001
Monotherapy
| Outcome | HR (95% CI) | |
|---|---|---|
| CKD and mortality (adjusted for known PAH prognostic factors), independently of aetiologyCKDvs No CKD | 1.83 (1.19–2.80) | |
| 0.20.5125Hazard ratio (log scale) |
- Chronic kidney disease (CKD) and mortality (adjusted for known PAH prognostic factors), independently of aetiology: only CKD of seven comorbidities analysed associated with poor prognosis
Monotherapy
- CTD-PAH received monotherapy more frequently
Original publication
New phenotype and outcomes in connective tissue disease-associated pulmonary arterial hypertension: relevance of age-related comorbidities. Rheumatology (Oxford). Published 27 February 2026. doi:10.
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