Long-term prognosis of patients with systemic lupus erythematosus-associated pulmonary arterial hypertension: CSTAR-PAH Cohort Study
European Respiratory Journal Jan 17, 2019
Qian J, et al. - In this multicenter prospective cohort, researchers identified the long-term clinical results and prognostic factors of patients with SLE-associated PAH confirmed by right heart catheterisation. A total of 310 patients were registered in 14 PAH centers. They identified baseline serositis, 6-minute walking distance >380 m and cardiac index ≥2.5 L·min−1×m2 as independent prognostic factors of treatment goal achievement (TGA), defined as an integrated outcome. Findings suggested an association of TGA with long-term survival, which supports the treat-to-target strategy in SLE-associated PAH. In patients with SLE-associated PAH, baseline heart function predicted both survival and treatment goal achievement. They observed that patients with baseline serositis tended to benefit from intensive immunosuppressive therapy and have a better clinical result. In patients with SLE-associated PAH, TGA was identified as a positive predictor of survival.
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