Objectives To summarize clinical characteristics and explore risk factors of pulmonary arterial hypertension (PAH) in patients with primary Sjögren disease (pSjD). Methods We retrospectively collected the clinical data of 190 pSjD inpatients, who were stratified into groups with PAH (pSjD-PAH) and without PAH (pSjD-non-PAH). The differences of clinical manifestations, laboratory tests, EULAR Sjögren’s Syndrome Disease Activity Index (ESSDAI) score, and transthoracic echocardiography (TTE) parameters between two groups were compared. A subgroup analysis was performed in pSjD-PAH patients according to pericardial effusion (PE) status. Results A total of 32 pSjD patients with PAH and 158 pSjD patients without PAH were analyzed. Patients in the pSjD-PAH group were older, with a higher incidence of mild interstitial lung disease (ILD) and diabetes (p < 0.05). They also exhibited higher levels of leukocytes, red blood cell distribution width (RDW), total bilirubin (TBIL), γ-glutamyltransferase (γ-GT), lactate dehydrogenase (LDH), and uric acid (UA) (p < 0.05). Logistic regression analysis identified mild ILD, age, and TBIL as independent risk factors for PAH in patients with pSjD. A predictive model was constructed and internally validated. In the subgroup analysis, ESSDAI score was comparable (p > 0.05), whereas values of pulmonary artery systolic pressure (PASP), pulmonary artery pressure (PA), and right atrial diameter (RAD) were higher in the PE-positive subgroup (p < 0.05). Conclusions Mild ILD, age, and TBIL are identified as independent risk factors for PAH in patients with pSjD. PE formation in pSjD-PAH patients may be associated with elevated right-sided heart pressure. <Table Float="No" ID="Taba"> <tgroup cols="2"> <colspec align="left" colname="c1" colnum="1" /> <colspec align="left" colname="c2" colnum="2" /> <tbody> <row> <entry align="left" nameend="c2" namest="c1"> Key Points • Pulmonary arterial hypertension (PAH) is a common cause of death in patients with primary Sjögren disease (pSjD), and relevant studies remain limited. • PSjD patients with PAH are more likely to have pericardial effusion (PE), which may result from elevated right-sided heart pressure and subsequent venous return obstruction. • Several risk factors were identified to enable early identification of pSjD-PAH patients. </entry> </row> </tbody> </tgroup> </Table>

The clinical characteristics and risk factors of pulmonary arterial hypertension in primary Sjögren disease: a single-center retrospective study

期刊论文
发表日期：

2026年7月14日

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Shuqi Zhuang,
Ling Zheng,
Xuan Wang,
Jincheng Pu,
Yuanyuan Liang,
Zhenzhen Wu,
Youwei Zhang,
Jianping Tang

摘要

Objectives

To summarize clinical characteristics and explore risk factors of pulmonary arterial hypertension (PAH) in patients with primary Sjögren disease (pSjD).

Methods

We retrospectively collected the clinical data of 190 pSjD inpatients, who were stratified into groups with PAH (pSjD-PAH) and without PAH (pSjD-non-PAH). The differences of clinical manifestations, laboratory tests, EULAR Sjögren’s Syndrome Disease Activity Index (ESSDAI) score, and transthoracic echocardiography (TTE) parameters between two groups were compared. A subgroup analysis was performed in pSjD-PAH patients according to pericardial effusion (PE) status.

Results

A total of 32 pSjD patients with PAH and 158 pSjD patients without PAH were analyzed. Patients in the pSjD-PAH group were older, with a higher incidence of mild interstitial lung disease (ILD) and diabetes (p < 0.05). They also exhibited higher levels of leukocytes, red blood cell distribution width (RDW), total bilirubin (TBIL), γ-glutamyltransferase (γ-GT), lactate dehydrogenase (LDH), and uric acid (UA) (p < 0.05). Logistic regression analysis identified mild ILD, age, and TBIL as independent risk factors for PAH in patients with pSjD. A predictive model was constructed and internally validated. In the subgroup analysis, ESSDAI score was comparable (p > 0.05), whereas values of pulmonary artery systolic pressure (PASP), pulmonary artery pressure (PA), and right atrial diameter (RAD) were higher in the PE-positive subgroup (p < 0.05).

Conclusions

Mild ILD, age, and TBIL are identified as independent risk factors for PAH in patients with pSjD. PE formation in pSjD-PAH patients may be associated with elevated right-sided heart pressure.

Key Points

• Pulmonary arterial hypertension (PAH) is a common cause of death in patients with primary Sjögren disease (pSjD), and relevant studies remain limited.

• PSjD patients with PAH are more likely to have pericardial effusion (PE), which may result from elevated right-sided heart pressure and subsequent venous return obstruction.

• Several risk factors were identified to enable early identification of pSjD-PAH patients.