Background: Prior studies indicated improved survival in systemic sclerosis-associated pulmonary arterial hypertension (PAH) patients, but trends in systemic lupus erythematosus-associated PAH (SLE-PAH) survival remained unclear. Methods: Analyzing SLE-PAH patients from the nationwide CSTAR-PAH cohort, we divided them into two cohorts: A (2011-2016) and B (2016-2021), based on baseline right heart catheterization dates. We compared clinical characteristics, mortality, and treatment outcomes between these cohorts and with idiopathic PAH (IPAH) patients. Results: We enrolled 610 and 104 patients with SLE-PAH and IPAH, respectively. Patients with SLE-PAH were younger, had a higher proportion of low-risk patients, and had a significantly higher 10-year survival rate than those with IPAH (66.6% vs. 44.1%, p < 0.001). Cohort B had a longer 6-min walk distance, lower mean pulmonary arterial pressure and pulmonary vascular resistance, a better-preserved cardiac index, and less right ventricular dilation than cohort A. More patients in cohort B received intensive immunosuppressant- and PAH-targeted therapies. The 5-year survival rate was significantly higher in cohort B (88.1% vs. 77.5%, p = 0.006). Reaching low-risk profile of PAH (hazard ratio [HR] 0.34, 95% confidence interval [CI] 0.15-0.79, p = 0.012) and reaching lupus low-disease-activity state (HR 0.33, 95% CI 0.14-0.82, p = 0.016) were independent predictors of survival. The rate of achieving low-risk profile for PAH was considerably higher in patients initially treated with intensive immunosuppressive and dual-PAH-targeted therapies. Conclusions: Over the last decade in China, the clinical characteristics of patients with SLE-PAH have evolved and survival has improved. Early PAH detection and dual treatment-to-target strategies for both PAH and SLE have contributed to this improvement in survival.

The authors have declared no competing interest.

This study was supported by the Chinese National Key Technology R&D Program, Ministry of Science and Technology (2021YFC2501300, 2017YFC0907600), Beijing Municipal Science & Technology Commission (No. Z201100005520022, 23, 25-27), CAMS Innovation Fund for Medical Sciences (CIFMS) (2021-I2M-1-005, 2019-12M-2-008), and National High Level Hospital Clinical Research Funding (2022-PUMCH-B-013, C-002, D-009, A-228).

I confirm all relevant ethical guidelines have been followed, and any necessary IRB and/or ethics committee approvals have been obtained.

Yes

The details of the IRB/oversight body that provided approval or exemption for the research described are given below:

Ethical approval was granted by by the Institutional Review Board of Peking Union Medical College Hospital (PUMCH) (JS-2038).

I confirm that all necessary patient/participant consent has been obtained and the appropriate institutional forms have been archived, and that any patient/participant/sample identifiers included were not known to anyone (e.g., hospital staff, patients or participants themselves) outside the research group so cannot be used to identify individuals.

Yes

I understand that all clinical trials and any other prospective interventional studies must be registered with an ICMJE-approved registry, such as ClinicalTrials.gov. I confirm that any such study reported in the manuscript has been registered and the trial registration ID is provided (note: if posting a prospective study registered retrospectively, please provide a statement in the trial ID field explaining why the study was not registered in advance).

Yes

I have followed all appropriate research reporting guidelines, such as any relevant EQUATOR Network research reporting checklist(s) and other pertinent material, if applicable.

Yes

Data collected for the CSTAR-PAH cohort, including de-identified individual participant data and a data dictionary defining each field in the set, will be available to researchers who will provide a methodologically sound and ethically approved proposal within 24 months after the publication of this article. Data sharing requests should be submitted to the corresponding authors.