Background Previous studies have described improved survival in systemic sclerosis (SSc) associated pulmonary arterial hypertension (PAH), yet it is unclear whether survival of systemic lupus erythematosus (SLE) associated PAH has also improved.

Methods A multi-center cohort of SLE-PAH patients diagnosed by right heart catheterization (RHC) was established and divided into cohort A (2011.6-2016.5) and cohort B (2016.6-2021.5) according to the date of their baseline RHC. Another single-center cohort of idiopathic pulmonary arterial hypertension (IPAH) was consecutively recruited as control to describe the baseline characteristic and survival of SLE-PAH patients simultaneously. Disease characteristics and all-cause mortality were compared between cohort A and B. Multivariable cox regression was used to analyze association between treatment goal achievement and survival.

Results A total of 610 SLE-PAH and 104 IPAH patients were enrolled. Overall, SLE-PAH patients were younger, had lower NT-proBNP level, better function status, better hemodynamic, and higher overall survival than IPAH (81.2% vs 56.0%, p<0.001). Compared with cohort A, patients in cohort B showed lower mPAP and PVR, higher CI, and were more likely to receive extensive immunosuppressants and PAH-targeted medication. 5-year survival rate was also higher in cohort B than cohort A (88.1% vs 72.9%, p=0.01). In multivariable Cox regression, treatment goal achievement of PAH (HR 0.31, 95%CI 0.12–0.81, p=0.017) and reaching lupus low disease activity state (LLDAS) (HR 0.23, 95%CI 0.08–0.67, p=0.007) were independently associated with a lower mortality.

Conclusions This largest muti-center prospective SLE-PAH cohort showed that survival has improved significantly for SLE-PAH in the last 5 years, and for the first time, demonstrated achieving LLDAS for SLE is associated with reduced mortality for SLE-PAH patients.