title: Chinese SLE Treatment and Research group (CSTAR) registry: II. Prevalence and risk factors of pulmonary arterial hypertension in Chinese patients with systemic lupus erythematosus.
abstract: OBJECTIVE
  To estimate the prevalence of pulmonary arterial hypertension (PAH) and risk factors for PAH in patients registered in the Chinese SLE Treatment and Research group (CSTAR) database, the first online registry of Chinese patients with systemic lupus erythematosus (SLE).
  
  
  METHODS
  A prospective cross-sectional study of patients with SLE was conducted using the CSTAR registry. Resting transthoracic echocardiography was used to estimate pulmonary artery pressure (PAP); PAH was defined as systolic PAP (PASP)≥40 mmHg. Patients with interstitial lung disease, valvular disease or cardiomyopathy were excluded because of disease influence on PAP. We explored potential risk factors for PAH including patient characteristics, organ involvement, laboratory findings and SLE disease activity.
  
  
  RESULTS
  Of 1934 patients with SLE, 74 had PASP with 54.2±17.1 (40,106) mmHg and were diagnosed with probable PAH. The incidences of lupus nephritis, pleuritis, pericarditis, hypocomplementemia, anti-SSA, and anti-ribonucleoprotein (RNP) were significantly higher in patients with PAH than in those without (p<0.05). SLE disease activity was significantly higher in patients with PAH than in unaffected patients (p<0.05). Multivariate analysis indicated that pericarditis (odds ratio (OR)=4.248), pleuritis (OR=3.061) and anti-RNP (OR=2.559) were independent risk factors for PAH in patients with SLE (p<0.05).
  
  
  CONCLUSIONS
  The possible prevalence of PAH was 3.8% in Chinese patients with SLE in the CSTAR registry. The significant association of pericarditis, pleuritis and anti-RNP positivity with PAH suggests that higher disease activity and vasculopathy may both contribute to the development of PAH in SLE, which need be treated aggressively to improve prognosis.
authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, China.
authors.forename: M
authors.initials: M
authors.lastname: Li
authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, China.
authors.forename: Q
authors.initials: Q
authors.lastname: Wang
authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, China.
authors.forename: J
authors.initials: J
authors.lastname: Zhao
authors.affiliation: Department of Rheumatology, the Affiliated Hospital of Bengbu Medical College, China.
authors.forename: Z
authors.initials: Z
authors.lastname: Li
authors.affiliation: Department of Rheumatology, the Fourth People's Hospital of Shenzhen Affiliated to Guangdong Medical College, China.
authors.forename: Z
authors.initials: Z
authors.lastname: Ye
authors.affiliation: Department of Rheumatology, Beijing Children Hospital Affiliated to Capital Medical University, China.
authors.forename: C
authors.initials: C
authors.lastname: Li
authors.affiliation: Department of Rheumatology, the Second Hospital of Shanxi Medical University, China.
authors.forename: X
authors.initials: X
authors.lastname: Li
authors.affiliation: Department of Clinical Immunology and Rheumatology, Xijing Hospital affiliated to the Fourth Military Medical University, China.
authors.forename: P
authors.initials: P
authors.lastname: Zhu
authors.affiliation: Department of Rheumatology, Beijing Tongren Hospital affiliated to Capital Medical University, China.
authors.forename: Z
authors.initials: Z
authors.lastname: Wang
authors.affiliation: Department of Rheumatology, Beijing Chao-Yang Hospital affiliated to Capital Medical University, China.
authors.forename: Y
authors.initials: Y
authors.lastname: Zheng
authors.affiliation: Department of Rheumatology, Anhui Provincial Hospital, China.
authors.forename: X
authors.initials: X
authors.lastname: Li
authors.affiliation: Department of Rheumatology, Jiangsu Provincial People's Hospital, China.
authors.forename: M
authors.initials: M
authors.lastname: Zhang
authors.affiliation: Department of Cardiology, Peking Union Medical College Hospital, China.
authors.forename: Z
authors.initials: Z
authors.lastname: Tian
authors.affiliation: Department of Cardiology, Peking Union Medical College Hospital, China.
authors.forename: Y
authors.initials: Y
authors.lastname: Liu
authors.affiliation: Pulmonary Vascular Center, Fu Wai Cardiovascular Hospital, China.
authors.forename: J
authors.initials: J
authors.lastname: He
authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, China.
authors.forename: F
authors.initials: F
authors.lastname: Zhang
authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, China.
authors.forename: Y
authors.initials: Y
authors.lastname: Zhao
authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, China zengxfpumc@gmail.com.
authors.forename: X
authors.initials: X
authors.lastname: Zeng
mesh_terms: D000293:Adolescent; D000328:Adult; D062186:Arterial Pressure; D044466:Asian People; D016009:Chi-Square Distribution; D002681:China / Q000453:epidemiology; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000208:ethnology* / Q000503:physiopathology; D015994:Incidence; D016015:Logistic Models; D008180:Lupus Erythematosus, Systemic / Q000097:blood / Q000175:diagnosis / Q000208:ethnology* / Q000276:immunology; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D016017:Odds Ratio; D015995:Prevalence; D011446:Prospective Studies; D011651:Pulmonary Artery / Q000503:physiopathology; D012042:Registries; D018570:Risk Assessment; D012307:Risk Factors; D013997:Time Factors; D055815:Young Adult
keywords: Prevalence; pulmonary arterial hypertension; risk factors; systemic lupus erythematosus