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Clinical phenotypes, hemodynamic characteristics and prognosis of Chinese patients with systemic sclerosis-associated precapillary pulmonary hypertension: a retrospective study

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Abstract

Introduction

Systemic sclerosis (SSc) is associated with interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH). This study aimed to explore the prevalence, clinical features, hemodynamic characteristics and prognosis of different severity of ILD in a cohort of patients with SSc-associated precapillary pulmonary hypertension (SSc-PH) and investigate the differences between SSc-PAH and idiopathic pulmonary arterial hypertension (IPAH) patients.

Method

SSc-PH patients and IPAH patients, admitted to Shanghai Pulmonary Hospital (August 1, 2008–January 31, 2020) and diagnosed by right-sided heart catheterization (RHC) or echocardiography, were retrospectively included. SSc-PH patients had a baseline chest high-resolution computed tomography (HRCT), and PH classification was based on the extent of ILD. Clinical, pulmonary function, hemodynamic characteristics and survival data were extracted.

Results

The study included 45 SSc-PH patients (60% had coexisting ILD and 77.8% were SSc-Group 1 PH/SSc-PAH [without ILD or with mild ILD], 22.2% were SSc-Group 3 PH/SSc-PH with severe ILD) and 52 IPAH patients. SSc-PH with ILD had lower arterial oxygen partial pressure (PaO2) than those without ILD. Hemodynamic characteristics and survival rates were comparable between SSc-PAH with mild ILD and those without ILD. SSc-Group 3 PH had lower pulmonary vascular resistance (PVR) and more severe restrictive ventilatory dysfunction than SSc-Group 1 PH, but the survival rate was equally poor. SSc-PAH had a poorer prognosis than IPAH patients despite the better hemodynamic characteristics.

Conclusions

ILD was common in SSc-PH patients. Careful phenotyping of PH in SSc-PH patients is very important as it is imperative to recognize its impact on clinical course, treatment and survival.

Key Points

• ILD was common in Chinese SSc-PH patients.

• SSc-PH patients with ILD had lower PaO 2 than those without ILD.

• Hemodynamic characteristics and survival rates were similar in SSc-PAH patients with mild ILD and those without ILD.

• Patients in SSc-Group 3 PH had lower pulmonary vascular resistance (PVR) and more severe restrictive ventilatory dysfunction than those in SSc-Group 1 PH, but the survival rate was equally poor. SSc-PAH patients had a poorer prognosis than IPAH patients despite their better hemodynamic characteristics.

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Funding

This study was supported by the Programme of the National Natural Science Foundation of China (81900050, 81870042 and 81700045), the Youth Project of Shanghai Municipal Commission of Health and Family Planning (20174Y0143), the National Science and Technology Information System of the People’s Republic of China (2018YFC1313603) and the National Health and Family Planning Commission of the People’s Republic of China (2015BAI12B10). The funders had no role in the study design, data collection and analysis, decision to publish or preparation of the manuscript.

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Correspondence to Su-Gang Gong, Lan Wang or Qin-Hua Zhao.

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This study was approved by the ethics review committee of Shanghai Pulmonary Hospital, which waived the requirement for informed consent due to the retrospective study design.

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Ping Yuan and Yi-Liang Su are co-first authors.

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Yuan, YP., Yuan, P., Su, YL. et al. Clinical phenotypes, hemodynamic characteristics and prognosis of Chinese patients with systemic sclerosis-associated precapillary pulmonary hypertension: a retrospective study. Clin Rheumatol 41, 1675–1686 (2022). https://doi.org/10.1007/s10067-021-06016-8

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