Long-Term Prognosis of Patients with Systemic Lupus Erythematosus-Associated Pulmonary Arterial Hypertension: CSTAR-PAH Cohort Study

Junyan Qian; Mengtao Li; Xiao Zhang; Qian Wang; Jiuliang Zhao; Zhuang Tian; Wei Wei; Xiaoxia Zuo; Miaojia Zhang; Ping Zhu; Shuang Ye; Wei Zhang; Yi Zheng; Wufang Qi; Yang Li; Zhuoli Zhang; Feng Ding; Jieruo Gu; Yi Liu; Yanhong Wang; Xiaofeng Zeng

doi:10.1183/13993003.00081-2018

Abstract

Aims: This study aimed to identify the long-term clinical outcomes and prognostic factors of patients with SLE-associated PAH confirmed by right heart catheterisation.

Methods and Results: A multicenter prospective cohort of SLE-associated PAH was established. Baseline and follow-up records were collected. The primary end point was death. The secondary exploratory end point was treatment goal achievement (TGA), defined as an integrated outcome. In total, 310 patients were enrolled from 14 PAH centers. 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. Baseline serositis, 6-minute walking distance >380 m and cardiac index ≥2.5 L·min⁻¹×m² were identified as independent prognostic factors of TGA. Patients with baseline serositis were more likely to reach TGA after intensive immunosuppressive therapy. TGA was identified as a positive predictor of survival in patients with SLE-associated PAH.

Conclusion: TGA was associated with long-term survival, which supports the treat-to-target strategy in SLE-associated PAH. Baseline heart function predicted both survival and treatment goal achievement in patients with SLE-associated PAH. Patients with serositis at baseline tended to benefit from intensive immunosuppressive therapy and have a better clinical outcome.

Footnotes

This manuscript has recently been accepted for publication in the European Respiratory Journal. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.

Conflict of interest: Dr. Ding has nothing to disclose.

Conflict of interest: Dr. Gu has nothing to disclose.

Conflict of interest: Dr. Zhao has nothing to disclose.

Conflict of interest: Dr. Qian has nothing to disclose.

Conflict of interest: Dr. Li has nothing to disclose.

Conflict of interest: Dr. Zhang has nothing to disclose.

Conflict of interest: Dr. Zhu has nothing to disclose.

Conflict of interest: Dr. Wang has nothing to disclose.

Conflict of interest: Dr. Ye has nothing to disclose.

Conflict of interest: Dr. Wei has nothing to disclose.

Conflict of interest: Dr. Zhang has nothing to disclose.

Conflict of interest: Dr. Qi has nothing to disclose.