Researchers discovered that risk assessment in patients with pulmonary arterial hypertension (PAH) associated with systemic lupus erythematosus (SLE) has significant prognostic value, according to a study published in SAGE Journals. 

PAH is a potentially deadly complication of SLE, being 1 of the top 3 causes of death in patients with SLE. SLE has surpassed Sjorgen’s syndrome and systemic sclerosis as the main connective tissue disease (CTD) associated with PAH. However, risk assessment has not been thoroughly characterized in SLE-associated PAH. 

Studies have demonstrated that a multidimensional risk stratification system for PAH holds prognostic value. In SLE, inflammation is a major driver of PAH; the authors of the study hence proposed that any risk assessment of SLE-associated PAH should include serositis as an inflammation indicator.

Continue Reading

“The aim of this study was to evaluate the prognostic value of the multivariable PAH risk-assessment system in SLE-associated PAH and to explore the role of inflammation in the risk assessment,” Wang and colleagues wrote. 

Read more about PAH etiology 

The research team hence recruited 305 patients with SLE-associated PAH who visited 14 qualified CTD-associated PAH referral centers in China. To be eligible for this study, participants must have been diagnosed with SLE according to the 2012 Systemic Lupus International Collaborating Clinics criteria, as well as PAH as defined by mean pulmonary arterial pressure of at least 25 mmHg at rest, pulmonary arterial wedge pressure of at least 15 mmHg, and pulmonary vascular resistance of more than 3 Wood units. 

The participants were then assessed according to multiple parameters—such as 6-minute walking distance, brain natriuretic peptide, N-terminal pro B-type natriuretic peptide, and WHO functional classification—and classified according to their level of risk: low, intermediate, and high. 

The results demonstrated that 42.0% of participants were classified as low-risk, 51.8% were classified as intermediate-risk, and 6.2% were classified as high-risk. The 1-year survival rates of the groups were 98.4%, 88.6%, and 78.9%, respectively, while the 5-year survival rates were 92.9%, 73.0%, and 47.5%, respectively. 

In addition, the authors of the study discovered that patients with serositis at baseline had significantly higher risk group improvement compared to those without baseline serositis, suggesting that patients with baseline serositis benefited from the initiation of immunosuppressive therapy. 

“Risk assessment has significant value in discriminating prognosis at both study baseline and the first follow-up assessment,” the authors of the study concluded. “Further investigation into the combination of SLE disease activity and PAH assessment for patients with SLE-associated PAH is urgently needed.” 


Wang Q, Qian J, Li M, et al. Risk assessment in systemic lupus erythematosus-associated pulmonary arterial hypertension: CSTAR-PAH cohort studyTher Adv Chronic Dis. 2022;13:20406223221112528. doi:10.1177/20406223221112528