Background and aims Systemic lupus erythematosus (SLE)-associated pulmonary arterial hypertension (PAH) is common in Asian countries, and the clinical outcome of patients with SLE-associated PAH is dramatically impaired. This study aimed to identify the long-term clinical outcomes and prognostic factors of patients with SLE-associated PAH confirmed by right heart catheterization (RHC).
Methods A multicenter cohort of SLE-associated PAH was established. Baseline and follow-up records were collected. The primary endpoint was death from any cause. The secondary experimental end point was treatment goal achievement (TGA).
Results Among the 310 patients enrolled from 14 PAH centres, 282 patients with confirmed mortality statuses were included in the survival analysis, 263 patients with complete follow-up data were included in the TGA study. The median follow-up was 24.0 months. The 1-, 3- and 5 year survival rates were 92.1%, 84.8% and 72.9%, respectively. The 1-, 3- and 5 year TGA rates were 31.5%, 53.6% and 62.7%, respectively. Serositis (HR=1.94, 95% CI: 1.26–3.00, p=0.003), 6MWD >380 m (HR=1.95, 95% CI: 1.14–3.31, p=0.014) and CI ≥2.5 L/min×m2 (HR=1.92, 95% CI: 1.16–3.19, p=0.012) were identified as independent prognostic factors of TGA.TGA within 5 years was identified as a factor associated with survival in patients with SLE-associated PAH.
Conclusions TGA was associated with the long-term survival, which supports and provides evidence to the treat-to-target strategy in SLE-associated PAH. Early diagnosis, intervention and heart function preservation are priorities for better long-term outcomes. PAH patients with high SLE activity may benefit from immunosuppressive therapy.
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