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Multicenter Study
. 2019 May;22(5):921-928.
doi: 10.1111/1756-185X.13478. Epub 2019 Feb 11.

Pulmonary arterial hypertension in systemic lupus erythematosus based on a CSTAR-PAH study: Baseline characteristics and risk factors

Na Zhang  1 , Mengtao Li  2   3 , Junyan Qian  2   3 , Qian Wang  2   3 , Jiuliang Zhao  2   3 , Zhenwen Yang  4 , Zhuang Tian  5 , Xiao Zhang  6 , Xiaoxia Zuo  7 , Miaojia Zhang  8 , Ping Zhu  9 , Shuang Ye  10 , Wei Zhang  11 , Yi Zheng  12 , Wufang Qi  13 , Yang Li  14 , Zhuoli Zhang  15 , Feng Ding  16 , Jieruo Gu  17 , Yi Liu  18 , Wei Wei  1 , Xiaofeng Zeng  2   3
Affiliations
Multicenter Study

Pulmonary arterial hypertension in systemic lupus erythematosus based on a CSTAR-PAH study: Baseline characteristics and risk factors

Na Zhang et al. Int J Rheum Dis. 2019 May.

Abstract

Aim: Pulmonary arterial hypertension (PAH) is a complex and devastating complication of systemic lupus erythematosus (SLE). We sought to describe the baseline characteristics of right heart catheterization (RHC)-confirmed SLE-associated PAH and identify risk factors for PAH in SLE patients.

Methods: A multicenter, cross-sectional study was conducted using the Chinese SLE Treatment and Research group (CSTAR) registry. Baseline data for patients with SLE-associated PAH and SLE patients without PAH were collected and compared. Risk factors for PAH among patients with SLE were identified.

Results: A total of 292 patients with SLE-associated PAH were enrolled. RHC was used to reveal hemodynamic features, including mean pulmonary arterial pressure (46.2 ± 12.0 mm Hg), pulmonary arterial wedge pressure (7.84 ± 3.92 mm Hg), pulmonary vascular resistance (10.86 ± 5.57 Wood units), and cardiac index (2.77 ± 0.91 L/min × m2 ). A multivariate logistic regression analysis showed that serositis (odds ratio [OR] = 5.524, 95% CI 3.605-8.465, P < 0.001), anti-ribonucleoprotein (RNP) antibody positivity (OR = 13.332, 95% CI 9.500-18.710, P < 0.001), and diffusion capacity of carbon monoxide in the lung (DLCO)/%Pred <70% (OR = 10.018, 95% CI 6.619-15.162, P < 0.001) were independent predictors of PAH. We recommend using transthoracic echocardiography (TTE) to perform early screening of SLE patients who have serositis, anti-RNP antibody positivity, or DLCO/%Pred <70%. RHC is suggested for patients suspected of having PAH. Once a diagnosis of SLE-PAH is confirmed, evaluation and treatment should immediately begin.

Conclusion: Overall, we recommend performing early screening using TTE in SLE patients with serositis, anti-RNP antibodies, or a DLCO/%Pred <70%, even for patients in a relatively stable condition according to SLE disease activity index.

Keywords: characteristics; pulmonary arterial hypertension; right heart catheterization; risk factors; systemic lupus erythematosus.

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