Risk factors and predictive model for fibrosis progression in patients with CT-defined usual interstitial pneumonia pattern_Chinese Journal of Respiratory and Critical Care Medicine

Authors：

YANG Shuqiao ¹ , WANG Yumei ¹ , WANG Yuanying ¹ , SAFEINA·DILIXIATI ¹ , SONG Yawen ^1,2 ,  YE Qiao ^1,2

1. Clinical Center for Interstitial Lung Diseases, Beijing Institute of Respiratory Medicine, Beijing ChaoYang Hospital, Capital Medical University, Beijing 100020, P.R. China;
2. Department of Occupational Medicine and Toxicology, Beijing Institute of Respiratory Medicine and Beijing Chao-Yang Hospital, Capital Medical University, Beijing 100020, P.R. China;

Corresponding?author：

YE Qiao, Email: yeqiao_chaoyang@sina.com

Keywords：

Usual interstitial pneumonia; pulmonary fibrosis progression; risk factor; risk prediction model

DOI：

10.7507/1671-6205.202510002

Video：

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Objective To identify risk factors for fibrosis progression and develop a predictive model in patients with usual interstitial pneumonia (UIP) pattern on CT. Methods We retrospectively enrolled 453 patients with CT-defined UIP or probable UIP, followed for one year. The study endpoint was either meeting progressive pulmonary fibrosis (PPF) criteria or completing one-year follow-up. Clinical features, pulmonary function, and laboratory data were collected. Independent risk factors were identified using logistic regression. Patients were randomly divided into training and validation cohorts at a 7:3 ratio. A nomogram was constructed in the training cohort using R and its performance and clinical utility were evaluated in the validation cohort. Results During one-year follow-up, 160 patients (35.3%) met PPF criteria. Multivariate analysis showed that higher baseline levels of CA19-9 and CA125, as well as the presence of pulmonary hypertension, were independent risk factors for pulmonary fibrosis progression, while a higher percentage of predicted forced vital capacity (FVC) and the presence of emphysema were protective factors. A nomogram model was constructed using these five variables, with the area under the curve (AUC) for predicting fibrosis progression being 0.854 in the training set and 0.817 in the validation set. Clinical decision curve analysis indicated that the model provided the greatest clinical benefit when the threshold probability was between 0.12 and 0.93. Conclusion A nomogram incorporating baseline CA19-9, CA125, FVC % predicted, pulmonary hypertension, and emphysema shows potential for predicting one-year fibrosis progression in UIP patients.

Citation： YANG Shuqiao, WANG Yumei, WANG Yuanying, SAFEINA·DILIXIATI, SONG Yawen, YE Qiao. Risk factors and predictive model for fibrosis progression in patients with CT-defined usual interstitial pneumonia pattern. Chinese Journal of Respiratory and Critical Care Medicine, 2025, 24(12): 859-865. doi: 10.7507/1671-6205.202510002 Copy

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3.	Qiu M, Jiang J, Nian X, et al. Factors associated with mortality in rheumatoid arthritis-associated interstitial lung disease: a systematic review and meta-analysis. Respir Res, 2021, 22(1): 264.
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12.	Kim K, Lee J, Jo YS. Factors for progressive pulmonary fibrosis in connective tissue disease-related interstitial lung disease. Ther Adv Respir Dis, 2023, 17: 17534666231212301.
13.	Cottin V, Hansell DM, Sverzellati N, et al. Effect of Emphysema Extent on Serial Lung Function in Patients with Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med, 2017, 196(9): 1162-1171.
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16.	Mohamed R, Sangani RG, Kamal KM, et al. Development of a local nomogram-based scoring system for predicting overall survival in idiopathic pulmonary fibrosis: A rural appalachian experience. Med Adv, 2024, 2(4): 336-348.
17.	Alhamad EH, Cal JG, Alrajhi NN, et al. Predictors of Mortality in Patients with Interstitial Lung Disease-Associated Pulmonary Hypertension. J Clin Med, 2020, 9(12): 3828.
18.	Dhont S, Zwaenepoel B, Vandecasteele E, et al. Pulmonary hypertension in interstitial lung disease: an area of unmet clinical need. ERJ Open Res, 2022, 8(4): 00272-02022.
19.	Matsuoka Y, Endo K, Kawamura Y, et al. Normal bronchial mucus contains high levels of cancer-associated antigens, CA125, CA19-9, and carcinoembryonic antigen. Cancer, 1990, 65(3): 506-510.
20.	Zheng M, Lou A, Zhang H, et al. Serum KL-6, CA19-9, CA125 and CEA are Diagnostic Biomarkers for Rheumatoid Arthritis-Associated Interstitial Lung Disease in the Chinese Population. Rheumatol Ther, 2021, 8(1): 517-527.
21.	Yang S, Zhan X, Wang J, et al. Serum Oncomarkers in Patients with MPO-ANCA-Positive Vasculitis: Diagnostic and Prognostic Predictive Values for Interstitial Lung Disease. Lung, 2022, 200(3): 331-338.
22.	Maher TM, Oballa E, Simpson JK, et al. An epithelial biomarker signature for idiopathic pulmonary fibrosis: an analysis from the multicentre PROFILE cohort study. Lancet Respir Med, 2017, 5(12): 946-955.
23.	Balestro E, Castelli G, Bernardinello N, et al. CA 19-9 serum levels in patients with end-stage idiopathic pulmonary fibrosis (IPF) and other interstitial lung diseases (ILDs): Correlation with functional decline. Chron Respir Dis, 2020, 17: 1479973120958428.
24.	Allen MR, Alevizos MK, Zhang D, et al. Performance of GAP and ILD-GAP models in predicting lung transplant or death in interstitial pneumonia with autoimmune features. Rheumatology (Oxford), 2024, 63(6): 1568-1573.
25.	Uyama M, Handa T, Uozumi R, et al. Prognostic value of a composite physiologic index developed by adding bronchial and hyperlucent volumes quantified via artificial intelligence technology. Respir Res, 2024, 25(1): 442.
26.	Clynick B, Corte TJ, Jo HE, et al. Biomarker signatures for progressive idiopathic pulmonary fibrosis. Eur Respir J, 2022, 59(3): 2101181.
27.	林希, 黃娜. 慢性阻塞性肺疾病急性加重期繼發肺部真菌感染的列線圖預測模型. 中國呼吸與危重監護雜志, 2024, 23(2): 77-85.

1. Ryerson CJ, Adegunsoye A, Piciucchi S, et al. Update of the International Multidisciplinary Classification of the Interstitial Pneumonias: An ERS/ATS Statement. Eur Respir J, 2025: 2500158.
2. Jacob J, Hirani N, van Moorsel CHM, et al. Predicting outcomes in rheumatoid arthritis related interstitial lung disease. Eur Respir J, 2019, 53(1): 1800869.
3. Qiu M, Jiang J, Nian X, et al. Factors associated with mortality in rheumatoid arthritis-associated interstitial lung disease: a systematic review and meta-analysis. Respir Res, 2021, 22(1): 264.
4. Ma R, Li S, Wang Y, et al. High-resolution computed tomography features of asbestosis versus fibrotic hypersensitivity pneumonitis: an observational study. BMC Pulm Med, 2022, 22(1): 207.
5. American Thoracic Society, European Respiratory Society. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med, 2002, 165(2): 277-304.
6. Zhang Y, Cen Z, Ding Q, et al. Prognostic significance of acute exacerbations and usual interstitial pneumonia in fibrotic interstitial lung disease. Sci Rep, 2025, 15(1): 21580.
7. Fainberg HP, Oldham JM, Molyneau PL, et al. Forced vital capacity trajectories in patients with idiopathic pulmonary fibrosis: a secondary analysis of a multicentre, prospective, observational cohort. Lancet Digit Health, 2022, 4(12): e862-e872.
8. Hwang YJ, Lee JK, Lee JH, et al. Validation of the gender, age, physiology model and other prognostic factors in interstitial lung disease patients with systemic autoimmune rheumatic disease. Sci Rep, 2025, 15(1): 24691.
9. 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. Am J Respir Crit Care Med, 2022, 205(9): e18-e47.
10. Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Respir J, 2015, 46(4): 903-975.
11. Ryerson CJ, Hartman T, Elicker BM, et al. Clinical features and outcomes in combined pulmonary fibrosis and emphysema in idiopathic pulmonary fibrosis. Chest, 2013, 144(1): 234-240.
12. Kim K, Lee J, Jo YS. Factors for progressive pulmonary fibrosis in connective tissue disease-related interstitial lung disease. Ther Adv Respir Dis, 2023, 17: 17534666231212301.
13. Cottin V, Hansell DM, Sverzellati N, et al. Effect of Emphysema Extent on Serial Lung Function in Patients with Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med, 2017, 196(9): 1162-1171.
14. Akagi T, Matsumoto T, Harada T, et al. Coexistent emphysema delays the decrease of vital capacity in idiopathic pulmonary fibrosis. Respir Med, 2009, 103(8): 1209-1215.
15. Fang YH, Hsieh YA, Chen YF, et al. Comparing survival outcomes of anti-fibrotic therapy for idiopathic pulmonary fibrosis with and without emphysema: a multi-center real-world study from Taiwan. BMC Pulm Med, 2025, 25(1): 401.
16. Mohamed R, Sangani RG, Kamal KM, et al. Development of a local nomogram-based scoring system for predicting overall survival in idiopathic pulmonary fibrosis: A rural appalachian experience. Med Adv, 2024, 2(4): 336-348.
17. Alhamad EH, Cal JG, Alrajhi NN, et al. Predictors of Mortality in Patients with Interstitial Lung Disease-Associated Pulmonary Hypertension. J Clin Med, 2020, 9(12): 3828.
18. Dhont S, Zwaenepoel B, Vandecasteele E, et al. Pulmonary hypertension in interstitial lung disease: an area of unmet clinical need. ERJ Open Res, 2022, 8(4): 00272-02022.
19. Matsuoka Y, Endo K, Kawamura Y, et al. Normal bronchial mucus contains high levels of cancer-associated antigens, CA125, CA19-9, and carcinoembryonic antigen. Cancer, 1990, 65(3): 506-510.
20. Zheng M, Lou A, Zhang H, et al. Serum KL-6, CA19-9, CA125 and CEA are Diagnostic Biomarkers for Rheumatoid Arthritis-Associated Interstitial Lung Disease in the Chinese Population. Rheumatol Ther, 2021, 8(1): 517-527.
21. Yang S, Zhan X, Wang J, et al. Serum Oncomarkers in Patients with MPO-ANCA-Positive Vasculitis: Diagnostic and Prognostic Predictive Values for Interstitial Lung Disease. Lung, 2022, 200(3): 331-338.
22. Maher TM, Oballa E, Simpson JK, et al. An epithelial biomarker signature for idiopathic pulmonary fibrosis: an analysis from the multicentre PROFILE cohort study. Lancet Respir Med, 2017, 5(12): 946-955.
23. Balestro E, Castelli G, Bernardinello N, et al. CA 19-9 serum levels in patients with end-stage idiopathic pulmonary fibrosis (IPF) and other interstitial lung diseases (ILDs): Correlation with functional decline. Chron Respir Dis, 2020, 17: 1479973120958428.
24. Allen MR, Alevizos MK, Zhang D, et al. Performance of GAP and ILD-GAP models in predicting lung transplant or death in interstitial pneumonia with autoimmune features. Rheumatology (Oxford), 2024, 63(6): 1568-1573.
25. Uyama M, Handa T, Uozumi R, et al. Prognostic value of a composite physiologic index developed by adding bronchial and hyperlucent volumes quantified via artificial intelligence technology. Respir Res, 2024, 25(1): 442.
26. Clynick B, Corte TJ, Jo HE, et al. Biomarker signatures for progressive idiopathic pulmonary fibrosis. Eur Respir J, 2022, 59(3): 2101181.
27. 林希, 黃娜. 慢性阻塞性肺疾病急性加重期繼發肺部真菌感染的列線圖預測模型. 中國呼吸與危重監護雜志, 2024, 23(2): 77-85.

Chinese Journal of Respiratory and Critical Care Medicine

Risk factors and predictive model for fibrosis progression in patients with CT-defined usual interstitial pneumonia pattern

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