Pulmonary arterial hypertension in systemic lupus erythematosus based on a CSTAR‐PAH study: Baseline characteristics and risk factors

Abstract Aim Pulmonary arterial hypertension (PAH) is a complex and devastating complication of systemic lupus erythematosus (SLE). We sought to describe the baseline characteristics of right heart catheterization (RHC)‐confirmed SLE‐associated PAH and identify risk factors for PAH in SLE patients. Methods A multicenter, cross‐sectional study was conducted using the Chinese SLE Treatment and Research group (CSTAR) registry. Baseline data for patients with SLE‐associated PAH and SLE patients without PAH were collected and compared. Risk factors for PAH among patients with SLE were identified. Results A total of 292 patients with SLE‐associated PAH were enrolled. RHC was used to reveal hemodynamic features, including mean pulmonary arterial pressure (46.2 ± 12.0 mm Hg), pulmonary arterial wedge pressure (7.84 ± 3.92 mm Hg), pulmonary vascular resistance (10.86 ± 5.57 Wood units), and cardiac index (2.77 ± 0.91 L/min × m 2 ). A multivariate logistic regression analysis showed that serositis (odds ratio [OR] = 5.524, 95% CI 3.605‐8.465, P < 0.001), anti‐ribonucleoprotein (RNP) antibody positivity (OR = 13.332, 95% CI 9.500‐18.710, P < 0.001), and diffusion capacity of carbon monoxide in the lung (DLCO)/%Pred <70% (OR = 10.018, 95% CI 6.619‐15.162, P < 0.001) were independent predictors of PAH. We recommend using transthoracic echocardiography (TTE) to perform early screening of SLE patients who have serositis, anti‐RNP antibody positivity, or DLCO/%Pred <70%. RHC is suggested for patients suspected of having PAH. Once a diagnosis of SLE‐PAH is confirmed, evaluation and treatment should immediately begin. Conclusion Overall, we recommend performing early screening using TTE in SLE patients with serositis, anti‐RNP antibodies, or a DLCO/%Pred <70%, even for patients in a relatively stable condition according to SLE disease activity index.