M Li1, Q Wang1, J Zhao1, Z Li2, Z Ye3, C Li4, X Li9, P Zhu6, Z Wang7, Y Zheng8, X Li9, M Zhang10, Z Tian11, Y Liu11, J He12, F Zhang1, Y Zhao1, X Zeng13. 1. Department of Rheumatology, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, China. 2. Department of Rheumatology, the Affiliated Hospital of Bengbu Medical College, China. 3. Department of Rheumatology, the Fourth People's Hospital of Shenzhen Affiliated to Guangdong Medical College, China. 4. Department of Rheumatology, Beijing Children Hospital Affiliated to Capital Medical University, China. 5. Department of Rheumatology, the Second Hospital of Shanxi Medical University, China. 6. Department of Clinical Immunology and Rheumatology, Xijing Hospital affiliated to the Fourth Military Medical University, China. 7. Department of Rheumatology, Beijing Tongren Hospital affiliated to Capital Medical University, China. 8. Department of Rheumatology, Beijing Chao-Yang Hospital affiliated to Capital Medical University, China. 9. Department of Rheumatology, Anhui Provincial Hospital, China. 10. Department of Rheumatology, Jiangsu Provincial People's Hospital, China. 11. Department of Cardiology, Peking Union Medical College Hospital, China. 12. Pulmonary Vascular Center, Fu Wai Cardiovascular Hospital, China. 13. Department of Rheumatology, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, China zengxfpumc@gmail.com.
Abstract
OBJECTIVES: 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.
OBJECTIVES: 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: Pedro Anuardo; Monica Verdier; Natali W S Gormezano; Gabriela R V Ferreira; Gabriela N Leal; Alessandro Lianza; Juliana C O A Ferreira; Rosa M R Pereira; Nadia E Aikawa; Maria Teresa Terreri; Claudia S Magalhães; Simone Appenzeller; Maria Carolina Dos Santos; Silvana B Sachetti; Claudio A Len; Gecilmara S Pilleggi; Simone Lotufo; Eloisa Bonfá; Clovis A Silva Journal: Pediatr Cardiol Date: 2016-11-08 Impact factor: 1.655
Authors: Ji Li; Jing-Ran Ma; Zhi-Xing Sun; Jing-Jing Jiang; Yan-Qing Dong; Qian Wang; Hong-Mei Song Journal: Zhongguo Dang Dai Er Ke Za Zhi Date: 2017-06