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Two distinct clinical phenotypes of pulmonary arterial hypertension secondary to systemic lupus erythematosus
  1. Fangfang Sun1,
  2. Yunxia Lei2,
  3. Wanlong Wu1,
  4. Li Guo1,
  5. Kaiwen Wang1,
  6. Zhiwei Chen1,
  7. Wenwen Xu1,
  8. Xiaodong Wang1,
  9. Ting Li1,
  10. Xiao Zhang2,
  11. Shuang Ye1
  1. 1 Department of Rheumatology, Ren Ji Hospital South Campus, School of Medicine, Shanghai Jiaotong University, Shanghai, China
  2. 2 Department of Rheumatology, Guangdong General Hospital, Guangzhou, Guangzhou, China
  1. Correspondence to Dr Xiao Zhang, Department of Rheumatology, Guangdong General Hospital, Guangdong 510080, China; 13922255387{at}163.com; Professor Shuang Ye, Department of Rheumatology, School of Medicine, Ren Ji Hospital South Campus, Shanghai Jiao Tong University, Shanghai 200001, China; ye_shuang2000{at}163.com

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Pulmonary arterial hypertension (PAH) is a severe complication of systemic lupus erythematosus (SLE), and SLE-PAH is the most common connective tissue disease (CTD)-associated PAH in Chinese patients.1 The prognosis of SLE-PAH is poor, with 3-year survival rates varying from 45% to 88%.2 3 Due to the complexity and heterogeneity of the underlying disease, it is necessary to further differentiate among SLE-PAH patterns to better understand the disease and optimise its management.

Between 2011 and 2016, a derivation cohort (Shanghai Ren Ji Hospital, n=108) and a validation cohort (Guangdong General Hospital, n=87) of patients with SLE-PAH from two medical centres were included. Patients with SLE-PAH were diagnosed based on right heart catheterisation or echocardiography (peak tricuspid regurgitation velocity >3.4 m/s), and those with left heart disease, pulmonary thrombosis and lung diseases were excluded.4 Based on the baseline clinical manifestations and laboratory findings at the time of the diagnosis of PAH, two distinct clusters were identified and validated by multiple correspondence analysis and k-means clustering. …

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