Abstract
OBJECTIVE: 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). METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.