Clinical, serological and haemodynamic factors associated with poor outcomes in systemic lupus erythematosus-associated pulmonary arterial hypertension: a multicentre retrospective study from Korea.

Abstract

To identify factors associated with adverse outcomes in Korean patients with systemic lupus erythematosus-associated pulmonary arterial hypertension (SLE-PAH) confirmed by right heart catheterization (RHC). This multicentre retrospective study included 68 patients with SLE-PAH diagnosed by RHC at eight tertiary centres. Baseline demographic, clinical, laboratory and haemodynamic data were collected, along with SLE Disease Activity Index (SLEDAI) scores and PAH-related parameters. Primary endpoint was a composite outcome of worsening PAH symptoms requiring treatment escalation, lung transplantation, death or hospitalization. Univariable and multivariable logistic regression analyses were performed to identify independent predictors. Of 68 patients, 35 (51.3%) experienced composite outcomes. Compared with those without composite outcomes, patients with composite outcomes had significantly shorter baseline 6-min walk distances (P = 0.024), higher tricuspid regurgitation velocity (P = 0.032), higher right ventricular systolic pressure (P = 0.035), higher mean pulmonary arterial pressure (mPAP) (P < 0.001), higher mean physician global assessment scores (P = 0.001), higher mean SLEDAI scores (P = 0.005) and more frequent use of combination PAH therapy (P = 0.033). In multivariable analysis, elevated mPAP (odds ratio (OR) 5.401; 95% confidence interval (CI) 1.129-25.837, P = 0.035), higher SLEDAI (OR 5.495; 95% CI 1.041-29.012, P = 0.045) and combination PAH therapy (OR 1.695; 95% CI 1.015-3.267, P = 0.017) remained independent predictors of composite outcomes. Elevated mPAP, active lupus and use of combination PAH therapy were independently associated with poor outcomes in SLE-PAH, highlighting the relevance of comprehensive haemodynamic assessment and careful management of lupus activity.