Chinese SLE Treatment and Research group (CSTAR) registry: II. Prevalence and risk factors of pulmonary arterial hypertension in Chinese patients with systemic lupus erythematosus

Li, M; Wang, Q; Zhao, Jiuliang; Li, Z; Ye, Zhen-Nan; Li, C; Li, X; Zhu, Ping; Wang, Z; Zheng, Yi; Zhang, M; Tian, Zhuang; Liu, Y; He, Jieyu; Zhang, F; Zhao, Yan; Zeng, Xiaofeng

doi:10.1177/0961203314527366

Cited by 78 publications

(91 citation statements)

References 43 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…13,36–40 Data based on CSTAR has shown pericardial effusion, serositis, and anti-RNP antibody are independent risk factors in ECHO-based SLE-PAH patients, 19 and later analysis of auto-antibodies has found that there is relevance between anti-SSA antibody and PAH. 41 …”

Section: Discussionmentioning

confidence: 99%

“…This group mimics SSc-PAH, in which irreversible vascular pathological changes, especially proliferation of endothelial cells and vascular smooth muscle cells, gradually lead to PAH during long disease duration. Anti-RNP antibody and Raynaud's phenomenon (RP) were believed to be associated with this subtype, 14,19,34,42 and RP was also proved as an indicator of disease severity. 36,38,42–44 Percentage of RP in our study was 64.5%, in accordance with previous published data; 45 however, we lack the data of the control group.…”

Section: Discussionmentioning

confidence: 99%

“…18 Data from Chinese SLE Treatment and Research Group (CSTAR) has shown that the PAH prevalence in SLE patients is 3.8%, 19 and a recent CTD-PAH study revealed that SLE was the most common underlying disease in Chinese CTD-PAH patients, 20 which suggested that a large SLE-PAH patient population existed all over the country. PUMCH has started SLE-PAH treatment for 8 years.…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Baseline Characteristics and Risk Factors of Pulmonary Arterial Hypertension in Systemic Lupus Erythematosus Patients

Huang

Liu

et al. 2016

Medicine

View full text Add to dashboard Cite

Peking Union Medical College Hospital (PUMCH) has started a single-center right heart catheterization (RHC)-based pulmonary arterial hypertension (PAH) study in systemic lupus erythematosus (SLE) since 2006. The baseline characteristics of these patients were described and the risk factor for PAH in lupus was identified.The demographic, clinical, laboratory, and treatment characteristics of SLE patients with PAH when they were registered were collected as the baseline data. A case-control study was conducted by taking the admitted SLE-non-PAH patients adjusted for age and gender in a 4:1 ratio during the same period as the controls. The associated variables were examined by binary multivariate logistic regression analysis to identify possible risk factors. A total of 111 RHC-confirmed SLE-PAH patients were enrolled, with the onset age of 34.6 ± 8.6 years old and the average SLE duration of 5 years. RHC revealed mPAP as 46.4 ± 11.4 mm Hg, CI as 2.7 ± 0.8 L/min × m2, and PVR as 10.5 ± 4.8 WU. 46% of patients were WHO Fc I–II. All patients were treated with immunosuppressive agents and 65% patients had PAH-targeted therapy. The case-control study had confirmed 2 independent risk factors previously published: pericardial effusion (OR = 21.290, P < 0.001) and anti-RNP antibody (OR = 12.399, P < 0.001). Meanwhile, 6 independent variables were discovered: baseline SLE duration (OR = 1.118, P = 0.007), interstitial lung disease (OR = 17.027, P < 0.001=, without acute rash (OR = 3.258, P = 0.019), anti-SSA antibody (OR = 4.836, P = 0.004), SLEDAI≤9 (OR = 26.426, P < 0.001), ESR≤20 mm/h (OR = 12.068, P < 0.001), and uric acid > 357 μmol/L (OR = 9.666, P < 0.001) to be associated with PAH in SLE patients.The PUMCH study has shown that SLE patients complicated with PAH are usually earlier diagnosed and have less disease severity than patients without PAH. The immunosuppressive therapy rate and the PAH target therapy rate were high, which is consistent with reports from Western countries. This study has confirmed that pericardial effusion and positive anti-RNP antibody are risk factors for SLE-associated PAH. Long SLE disease duration, the presence of interstitial lung disease, without acute skin rash, positive anti-SSA antibody, low SLEDAI and ESR, and high uric acid levels are also associated with PAH in SLE patients.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Baseline Characteristics and Risk Factors of Pulmonary Arterial Hypertension in Systemic Lupus Erythematosus Patients

Huang

Liu

et al. 2016

Medicine

View full text Add to dashboard Cite

show abstract

“…2). Based on the SLE patients registered in the CRDC, papers on basic demographic characteristics and first symptom in Chinese SLE patients [5], risk factors of pulmonary hypertension [8], correlations between autoantibodies and clinical manifestations [9], and factors related to fetal loss [10] have been published. The SLE patients in the study of Li et al showed differing primary symptoms [5], of which rash, arthritis, fever, cytopenia, and kidney disease were the most common.…”

Section: Resultsmentioning

confidence: 99%

CRDC: a Chinese rheumatology research platform

Tian

Zhang

et al. 2015

Clin Rheumatol

View full text Add to dashboard Cite

This review introduces the history of development, organizational structure, funding resources, data collection, and quality control of the Chinese Rheumatism Data Center (CRDC) and summarizes the collection of data. In 2009, Peking Union Medical College Hospital (PUMCH), together with several rheumatism centers, established the Chinese Systemic Lupus Erythematosus (SLE) Treatment and Research Group (CSTAR) to collect data on Chinese patients for the study of SLE disease characteristics. In 2011, CSTAR was extended with the formation of the CRDC at PUMCH with direction from the National Health and Family Planning Commission of the PRC. The CRDC currently includes 300 registration sites and 50 regional sites that have successively begun to collect data on 12 rheumatic diseases, including systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), ankylosing spondylitis (AS), systemic sclerosis, dermatomyositis, Takayasu arteritis, IgG4-related diseases, ANCA-associated vasculitis, gout, polyarteritis nodosa, unclassified systemic vasculitis, and Behcet disease. To date, 17,224 patients have been enrolled in the CRDC. Based on the SLE patients registered in the CRDC, papers investigating basic demographic characteristics and first symptom in Chinese SLE patients, risk factors of pulmonary hypertension, correlations between autoantibodies and clinical manifestations, and factors related to fetal loss have been published. The CRDC is a national registry that provides real-life data to improve clinical decision-making. At the same time, without additional work for the clinician, the CRDC is a powerful research database. The CRDC database provides sufficient information for Chinese clinical studies on rheumatology. Moreover, a mobile device application ensures convenient and efficient data collection without compromising data quality, thereby providing strong evidence-based data for the diagnosis and treatment of Chinese rheumatic patients.

show abstract

“…However, most patients in that study had overt right heart failure, which may have contributed to pleural effusion. In a recent analysis of a large Chinese cohort (n=1,934, 74 patients with PAH), pleuritis was an independent predictor of PAH in lupus and showed an odds ratio (OR) =3.06 (95% confidence interval [CI] =1.6–5.85) 18. Additional clinical manifestations with an independent predictive ability for PAH were interstitial lung disease (OR =17, 95% CI =3.6–80) and pericardial effusion (OR =21.3, 95% CI =4.1–110.6) 13.…”

Section: Predictive Factorsmentioning

confidence: 99%

Systemic lupus erythematosus and pulmonary arterial hypertension: links, risks, and management strategies

Tselios¹,

Gladman²,

Urowitz³

2016

OARRR

View full text Add to dashboard Cite

Systemic lupus erythematosus (SLE) is characterized by the second highest prevalence of pulmonary arterial hypertension (PAH), after systemic sclerosis, among the connective tissue diseases. SLE-associated PAH is hemodynamically defined by increased mean pulmonary artery pressure at rest (≥25 mmHg) with normal pulmonary capillary wedge pressure (≤15 mmHg) and increased pulmonary vascular resistance. Estimated prevalence ranges from 0.5% to 17.5% depending on the diagnostic method used and the threshold of right ventricular systolic pressure in studies using transthoracic echocardiogram. Its pathogenesis is multifactorial with vasoconstriction, due to imbalance of vasoactive mediators, leading to hypoxia and impaired vascular remodeling, collagen deposition, and thrombosis of the pulmonary circulation. Multiple predictive factors have been recognized, such as Raynaud’s phenomenon, pleuritis, pericarditis, anti-ribonuclear protein, and antiphospholipid antibodies. Secure diagnosis is based on right heart catheterization, although transthoracic echocardiogram has been shown to be reliable for patient screening and follow-up. Data on treatment mostly come from uncontrolled observational studies and consist of immunosuppressive drugs, mainly corticosteroids and cyclophosphamide, as well as PAH-targeted approaches with endothelin receptor antagonists (bosentan), phosphodiesterase type 5 inhibitors (sildenafil), and vasodilators (epoprostenol). Prognosis is significantly affected, with 1- and 5-year survival estimated at 88% and 68%, respectively.

show abstract

Chinese SLE Treatment and Research group (CSTAR) registry: II. Prevalence and risk factors of pulmonary arterial hypertension in Chinese patients with systemic lupus erythematosus

Cited by 78 publications

References 43 publications

Baseline Characteristics and Risk Factors of Pulmonary Arterial Hypertension in Systemic Lupus Erythematosus Patients

Baseline Characteristics and Risk Factors of Pulmonary Arterial Hypertension in Systemic Lupus Erythematosus Patients

CRDC: a Chinese rheumatology research platform

Systemic lupus erythematosus and pulmonary arterial hypertension: links, risks, and management strategies

Contact Info

Product

Resources

About