Improvements and challenges of long-term survival in patients with systemic lupus erythematosus-associated pulmonary arterial hypertension: A 10-year multi-center cohort study

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.