Background Chinese SLE treatment and Research Group (CSTAR) started a multi-center prospective cohort study recruiting SLE patients with pulmonary arterial hypertension (PAH) since 2006. This study aimed to investigate the validity of a multimensional risk assessment and the prognostic value of it in SLE-associated PAH.
Methods All SLE patients were fulfilled the 1997 revised ACR criteria. PAH was diagnosed based on ESC/ERS guidelines by right heart catheterization. The outcome was all-cause mortality. Two different methods of risk categorization were applied according to baseline data, including low-risk criteria number of none to four and mean score of 1 (low-risk), 2 (intermediate-risk) or 3 (high-risk). According to first follow-up, patients were further divided into increased risk, remained risk and decreased risk group. A prediction model was used to distinguish SLE-PAH from vasculitic and vasculopathic subtype, based on the time interval between the diagnosis of SLE and PAH and Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). KaplanMeier survival curves and Cox proportional hazards analysis were conducted.
Results 282 patients were enrolled. The 5 year survival of patients with none, one, two, three and four low risk criteria were 42.7%, 64.8%, 86.1%, 90.2% and 91.7%, respectively (HR=0.59, 95% CI 0.44–0.78, p<0.001). The 5 year survival of patients in low-risk, intermediate-risk and high-risk group were 92.3%, 60.4% and 50.0%, respectively (Log-rank, p=0.001). Notably, in low-risk group, patients with vasculitic subtype had better survival than those with vasculopathic subtype (Log-rank, p=0.044). The 5 year survival of patients with remained, decreased and increased risk were 65.4%, 88.1% and 23.8%, respectively (log-rank, p<0.001).
Conclusions Our study, for the first time, validated the prognostic value of risk stratification strategy at baseline and follow-up visit in patients with SLE-associated PAH. Patients are recommended to have a comprehensive evaluation on PAH and SLE at baseline and every follow-up visit. The SLE disease activity and systemic manifestations predict the phenotype of SLE-associated PAH, which also effect the long-term survival and need to be involved into risk assessment of SLE-associated PAH. Improving to low-risk group can be a future treatment target for SLE-associated PAH patients in clinical practice.
Funding Source(s): This work was supported by the Chinese National Key Research R and D Program [grant number 2017YFC0907601, 2017YFC0907602, 2017YFC0907603, 2008BAI59B02], and the Chinese National High Technology Research and Development Program, Ministry of Science and Technology [grant number 2012AA02A513].
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