Construction and validation of a risk prediction model for pulmonary arterial hypertension in patients with systemic sclerosis-related interstitial lung disease

Wang Xiaoran; Zhao Peishuo; Mei Qiong; Yan Li; Zhang Aili

doi:null

您当前的位置：

首页 >

文章列表页 >

Construction and validation of a risk prediction model for pulmonary arterial hypertension in patients with systemic sclerosis-related interstitial lung disease

更新时间：2026-10-27

- Construction and validation of a risk prediction model for pulmonary arterial hypertension in patients with systemic sclerosis-related interstitial lung disease
- Journal of Chongqing Medical University Pages: 1-9(2026)
- 作者机构：
  
  河北省人民医院呼吸与危重症学科，石家庄 050051
- 作者简介：
- 基金信息：
- DOI：
  CLC： R563.9
- Received：25 February 2026，
  
  Revised：2026-07-03，
  
  Accepted：20 August 2026，
  
  Published：27 October 2026
- 稿件说明：
移动端阅览
Wang Xiaoran,Zhao Peishuo,Mei Qiong,et al.Construction and validation of a risk prediction model for pulmonary arterial hypertension in patients with systemic sclerosis-related interstitial lung disease[J].Journal of Chongqing Medical University,
DOI：

Wang Xiaoran,Zhao Peishuo,Mei Qiong,et al.Construction and validation of a risk prediction model for pulmonary arterial hypertension in patients with systemic sclerosis-related interstitial lung disease[J].Journal of Chongqing Medical University, DOI：10.13406/j.cnki.cyxb..

摘要

目的

探讨系统性硬化症合并间质性肺病（systemic sclerosis with interstitial lung disease，SSc-ILD）患者并发肺动脉高压（pulmonary arterial hypertension，PAH）的独立危险因素，并基于这些因素构建一个可视化的列线图预测模型。

方法

本研究为单中心回顾性队列研究。收集并分析2017年1月至2025年10月在河北省人民医院风湿免疫科及呼吸与危重症学科确诊并接受治疗的118例SSc-ILD患者的临床资料、实验室检查、影像学资料及肺功能数据。根据超声心动图结果，将患者分为PAH组（

=40）和非PAH组（

=78）。采用单因素分析、LASSO回归及多因素Logistic回归分析筛选并发PAH的独立危险因素。基于回归分析结果构建列线图预测模型。通过绘制受试者工作特征（receiver operating characteristic，ROC）曲线评估模型的区分度，利用校准曲线检验评估模型的校准度，并采用决策曲线分析（decision curve analysis，DCA）评估该模型的临床实用性。

结果

多因素Logistic回归分析最终筛选出5个SSc-ILD并发PAH的独立危险因素：超声心动图中右心室舒张末期基底内径（right ventricular end-diastolic basal diameter，RV）（

=1.20，95%CI=1.05~1.37，

=0.007）、SSc评分（

=1.33，95%CI=1.12~1.57，

=0.001）、肺功能检查中一氧化碳弥散量占预计值百分比（diffusing capacity of the lung for carbon monoxide percentage of predicted，DLCO%）（

=0.93，95%CI=0.89~0.97，

0.001）、胸部CT有蜂窝影（

=3.45，95%CI=1.08~11.04，

=0.037）、红细胞沉降率（

=1.04，95%CI=1.01~1.07，

=0.005）。基于上述5个因素构建的列线图模型校准曲线与理想曲线拟合度较好，提示列线图模型具有较高的预测准确度；ROC曲线下面积为0.909，初步显示列线图模型具有良好区分度但需外部验证。

结论

RV、SSc评分、DLCO％、蜂窝影及红细胞沉降率是SSc-ILD患者并发PAH的独立危险因素，并基于上述因素构建列线图模型，其显示出良好的区分度与校准度，但因EPV值低于推荐阈值，存在过拟合风险，其区分度有待外部验证。本模型有助于临床早期、个体化风险评估，为制定前瞻性筛查及干预策略提供依据。

Abstract

Objective

To investigate the independent risk factors for pulmonary arterial hypertension（PAH） in patients with systemic sclerosis-related interstitial lung disease（SSc-ILD），and to construct a visualized nomogram prediction model based on these factors.

Methods

A single-center retrospective cohort study was conducted among 118 patients with SSc-ILD who were diagnosed and treated at Department of Rheumatology and Immunology and Department of Respiratory and Critical Care Medicine，Hebei General Hospital，from January 2017 to October 2025，and related data were analyzed，including clinical data，laboratory tests，imaging data，and pulmonary function data. According to the results of echocardiography，the patients were divided into PAH group with 40 patients and non-PAH group with 78 patients. The univariate analysis，the LASSO regression analysis，and the multivariable logistic regression analysis were used to identify independent risk factors for PAH. A nomogram prediction model was constructed based on the results of regression analyses. The receiver operating characteristic（ROC） curve was plotted to assess the discriminatory ability of the model，the calibration curve was used to assess its calibration，and decision curve analysis was used to assess its clinical practicability.

Results

The multivariable logistic regression analysis identified five independent risk factors for PAH in SSc-ILD，i.e.，

right ventricular end-diastolic basal diameter（RV） measured by echocardiography（odds ratio［

］=1.20，95%CI=1.05-1.37，

=0.007），SSc score（

=1.33，95%CI=1.12-1.57，

=0.001），diffusing capacity of the lung for carbon monoxide percentage of predicted（DLCO%） measured by pulmonary function tests（

=0.93，95%CI=0.89-0.97，

0.001），honeycomb shadow on chest CT（

=3.45，95%CI=1.08-11.04，

=0.037），and erythrocyte sedimentation rate（ESR）（

=1.04，95%CI=1.01-1.07，

=0.005）. The calibration curve of the nomogram model constructed based on these 5 factors fit well with the ideal curve，suggesting that the nomogram model has high prediction accuracy. The nomogram model had an area under the ROC curve of 0.909，suggesting that it has good discriminatory ability but requires external validation.

Conclusion

RV，SSc score，DLCO%，honeycomb shadow，and ESR are independent risk factors for concurrent PAH in patients with SSc-ILD，and the nomogram model constructed based on these factors has good discriminatory ability and calibration. However，given that the value of events per variable was below the recommended threshold，the model carries a potential risk of overfitting，and thus external validation is needed to confirm its discriminatory ability. This model can be used to facilitate early and individualized risk assessment in clinical practice，thereby providing a basis for formulating prospective screening and intervention strategies.

关键词

Keywords

references

Volkmann ER ， Andréasson K ， Smith V . Systemic sclerosis [J ] . Lancet ， 2023 ， 401 （ 10373 ）： 304 - 318 ．

Bukiri H ， Volkmann ER . Current advances in the treatment of systemic sclerosis [J ] . Curr Opin Pharmacol ， 2022 ， 64 ： 102211 ．

任亚飞，蒋亚男，谢　璠，等 . 系统性硬化症相关间质性肺疾病的机制研究 [J ] . 实用医学杂志， 2018 ， 34 （ 19 ）： 3307 - 3309 ．

Ren YF ， Jiang YN ， Xie F ， et al . Mechanism of interstitial lung disease associated with systemic sclerosis [J ] . J Pract Med ， 2018 ， 34 （ 19 ）： 3307 - 3309 ．

雷　玲，袁　婧，赵　铖，等 . 系统性硬化病合并肺动脉高压的临床特点及治疗效果分析 [J ] . 广西医科大学学报， 2019 ， 36 （ 8 ）： 1264 - 1269 ．

Lei L ， Yuan J ， Zhao C ， et al . The clinical characteristics and therapeutic effect in patients with systemic sclerosis-associated pulmonary arterial hypertension [J ] . J Guangxi Med Univ ， 2019 ， 36 （ 8 ）： 1264 - 1269 ．

van den Hoogen F ， Khanna D ， Fransen J ， et al . 2013 classification criteria for systemic sclerosis：an American college of rheumatology/European league against rheumatism collaborative initiative [J ] . Ann Rheum Dis ， 2013 ， 72 （ 11 ）： 1747 - 1755 ．

Raghu G ， Remy-Jardin M ， Richeldi L ， et al . Idiopathic pulmonary fibrosis（an update） and progressive pulmonary fibrosis in adults：an official ATS/ERS/JRS/ALAT clinical practice guideline [J ] . Am J Respir Crit Care Med ， 2022 ， 205 （ 9 ）： 18 - 47 ．

Raghu G ， Montesi SB ， Silver RM ， et al . Treatment of systemic sclerosis-associated interstitial lung disease：evidence-based recommendations. an official American thoracic society clinical practice guideline [J ] . Am J Respir Crit Care Med ， 2024 ， 209 （ 2 ）： 137 - 152 ．

Helmreich JE . Regression modeling strategies with applications to linear models，logistic and ordinal regression and survival analysis（2nd edition） [J ] . J Stat Soft ， 2016 ， 70 （ 2 ）： 1 - 3 ．

Marchetti M ， Motta F ， Tonutti A ， et al . Combined immunosuppressive therapy in systemic sclerosis-associated interstitial lung disease：Phenotypes mattere [J ] . Autoimmun Rev ， 2026 ， 25 （ 8 ）： 104092 .

Zhao HQ ， Chakinala MM ， Fallon MB ， et al . Endothelial heterogeneity in pulmonary hypertension [J ] . ATVB ， 2026 ， 46 （ 1 ）： 3 - 16 ．

朱玲娴，赵荫环，张　蕊，等 . 系统性硬化症发生肺动脉高压的独立危险因素分析 [J ] . 风湿病与关节炎， 2023 ， 12 （ 1 ）： 14 - 18 ．

Zhu LX ， Zhao YH ， Zhang R ， et al . Independent risk factors of pulmonary hypertension in systemic sclerosis [J ] . Rheumatism Arthritis ， 2023 ， 12 （ 1 ）： 14 - 18 ．

刘　莹，王晓武，董文鹏 . 炎症反应在肺动脉高压中的研究进展 [J ] . 实用医学杂志， 2016 ， 32 （ 24 ）： 4136 - 4139 ．

Liu Y ， Wang XW ， Dong WP . Research progress of inflammatory reaction in pulmonary hypertension [J ] . J Pract Med ， 2016 ， 32 （ 24 ）： 4136 - 4139 ．

Young A ， Vummidi D ， Visovatti S ， et al . Prevalence，treatment，and outcomes of coexistent pulmonary hypertension and interstitial lung disease in systemic sclerosis [J ] . Arthritis Rheumatol ， 2019 ， 71 （ 8 ）： 1339 - 1349 ．

梁　金，金承基，黄含音，等 . 心脏超声评估右心功能对结缔组织病相关间质性肺病继发肺动脉高压的预测价值 [J ] . 临床内科杂志， 2025 ， 42 （ 1 ）： 25 - 29 ．

Liang J ， Jin CJ ， Huang HY ， et al . Predictive value of right heart function in diagnosing pulmonary hypertension secondary to connective tissue disease-associated interstitial lung disease by echocardiography [J ] . J Clin Intern Med ， 2025 ， 42 （ 1 ）： 25 - 29 ．

Khanna D ， Furst DE ， Clements PJ ， et al . Standardization of the modified rodnan skin score for use in clinical trials of systemic sclerosis [J ] . J Scleroderma Relat Disord ， 2017 ， 2 （ 1 ）： 11 - 18 ．

Zheng BY ， Nevskaya T ， Baxter CA ， et al . Changes in skin score in early diffuse cutaneous systemic sclerosis are associated with changes in global disease severity [J ] . Rheumatology ， 2020 ， 59 （ 2 ）： 398 - 406 ．

Hikmat IH ， Ramdhini SS ， Soekersi H ， et al . Correlation between interstitial lung disease morphology scores based on high-resolution computed tomography chest and skin fibrosis degree based on modified rodnan’s skin score on systemic sclerosis [J ] . Acta Med Indones ， 2021 ， 53 （ 3 ）： 254 - 260 ．

Rujirawinitchai P ， Foocharoen C ， Mahakkanukrauh A ， et al . Correlation between the peak of skin thickness progression rate and onset of cardiopulmonary involvement in Thai systemic sclerosis patients [J ] . J Clin Med ， 2025 ， 14 （ 7 ）： 2281 ．

Rohman S ， Rakhma YH ， Friska Anggraini HS . The relationship between modified rodnan skin score（mRSS） with pulmonal hypertension and lung fibrosis in systemic sclerosis patients [J ] . Ina J Rheum ， 2021 ， 13 （ 1 ）： 483 - 485 ．

Le Gouellec N ， Duhamel A ， Perez T ， et al . Predictors of lung function test severity and outcome in systemic sclerosis-associated interstitial lung disease [J ] . PLoS One ， 2017 ， 12 （ 8 ）： e0181692 ．

Diamanti E ， Karava V ， Yerly P ， et al . Carbon monoxide diffusion capacity as a severity marker in pulmonary hypertension [J ] . J Clin Med ， 2021 ， 11 （ 1 ）： 132 ．

Sarı A ， Temiz Karadağ D ， Erdoğan M ， et al . Screening for pulmonary hypertension in connective tissue diseases：literature review and multidisciplinary consensus statement [J ] . Anatol J Cardiol ， 2025 ， 29 （ 11 ）： 599 - 607 ．

Zou RH ， Wallace WD ， Nouraie SM ， et al . Lower DLco% identifies exercise pulmonary hypertension in patients with parenchymal lung disease referred for dyspnea [J ] . Pulm Circ ， 2020 ， 10 （ 1 ）： 1 - 11 ．

Mirsaeidi M ， Barletta P ， Glassberg MK . Systemic sclerosis associated interstitial lung disease：new directions in disease management [J ] . Front Med ， 2019 ， 6 ： 248 ．

Sahani D ， Singh Y ， Chumber S ， et al . Correlation of HRCT scoring system with PFT parameters in interstitial lung disease associated with systemic sclerosis patients [J ] . Indian J Chest Dis Allied Sci ， 2022 ， 64 （ 1 ）： 21 - 27 ．

Cottin V ， Hirani NA ， Hotchkin DL ， et al . Presentation，diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases [J ] . Eur Respir Rev ， 2018 ， 27 （ 150 ）： 180076 ．

Pugashetti JV ， Khanna D ， Kazerooni EA ， et al . Clinically relevant biomarkers in connective tissue disease-associated interstitial lung disease [J ] . Immunol Allergy Clin North Am ， 2023 ， 43 （ 2 ）： 411 - 433 ．

Chatterjee S ， Perelas A ， Yadav R ， et al . Viewpoint：a multidisciplinary approach to the assessment of patients with systemic sclerosis-associated interstitial lung disease [J ] . Clin Rheumatol ， 2023 ， 42 （ 3 ）： 653 - 661 ．

Guiot J ， Henket M ， Gester F ， et al . Automated AI-based image analysis for quantification and prediction of interstitial lung disease in systemic sclerosis patients [J ] . Respir Res ， 2025 ， 26 （ 1 ）： 39 ．

Pendergrass SA ， Hayes E ， Farina G ， et al . Limited systemic sclerosis patients with pulmonary arterial hypertension show biomarkers of inflammation and vascular injury [J ] . PLoS One ， 2010 ， 5 （ 8 ）： e12106 ．

Iannone F ， Riccardi MT ， Guiducci S ， et al . Bosentan regulates the expression of adhesion molecules on circulating T cells and serum soluble adhesion molecules in systemic sclerosis-associated pulmonary arterial hypertension [J ] . Ann Rheum Dis ， 2008 ， 67 （ 8 ）： 1121 - 1126 ．

Michalska-Jakubus MM ， Rusek M ， Kowal M ， et al . Anti-endothelial cell antibodies are associated with apoptotic endothelial microparticles，endothelial sloughing and decrease in angiogenic progenitors in systemic sclerosis [J ] . Postepy Dermatol Alergol ， 2020 ， 37 （ 5 ）： 725 - 735 ．

王顺兵，郑建雄，廖　霞，等 . 系统性硬化症相关肺间质病变的临床特征及危险因素分析 [J ] . 四川医学， 2021 ， 42 （ 9 ）： 907 - 912 ．

Wang SB ， Zheng JX ， Liao X ， et al . Analysis of clinical characteristics and risk factors of lung interstitial disease associated with systemic sclerosis [J ] . Sichuan Med J ， 2021 ， 42 （ 9 ）： 907 - 912 ．

Jung E ， Suh CH ， Kim HA ， et al . Clinical characteristics of systemic sclerosis with interstitial lung disease [J ] . Arch Rheumatol ， 2018 ， 33 （ 3 ）： 322 - 327 ．

Views

下载量

CSCD

Alert me when the article has been cited

提交

Tools

Publicity Resources

Construction of a predictive model for the severity of obstructive sleep apnea in patients with type 2 diabetes mellitus based on estimated glucose disposal rate

Influencing factors for sarcopenia in elderly hospitalized patients and construction and validation of a risk prediction model

Construction and validation of a nomogram prediction model for new-onset atrial fibrillation during hospitalization in patients with acute ST-elevation myocardial infarction

Association of post-stroke peripheral immune status with prognosis in young and middle-aged patients with acute ischemic stroke

Electroacupuncture at Jiaji points inhibits nuclear factor-kappa B-induced neurotoxic A1 astrocyte differentiation in mice after spinal cord injury by regulating the phosphatidylinositol 3-kinase/protein kinase B/tumor necrosis factor-α signaling pathway

Related Author

Liu Shumin

Wei Zhenna

Zhang Wei

Chen Yun

Chen Lili

Qi Yanhuan

Zhou Yilin

Fan Qingqing

Related Institution

Department of Nursing，Shenzhen Hospital（Futian），Guangzhou University of Chinese Medicine

Department of Endocrinology，Shenzhen Hospital（Futian），Guangzhou University of Chinese Medicine

Department of General Practice，Chongqing University Central Hospital

Department of Geriatrics，The First Affiliated Hospital of Chongqing Medical University

Department of Health Statistics，College of Public Health，Chongqing Medical University

⁰