Prognostic significance of the usual interstitial pneumonia pattern in interstitial lung diseases

The usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography (HRCT) is a key diagnostic hallmark of idiopathic pulmonary fibrosis (IPF). However, its prevalence and prognostic significance in other fibrosing interstitial lung diseases (ILDs), such as fibrotic hypersensitivity pneumonitis (fHP) and fibrotic ILD associated with systemic sclerosis (fILD-SSc), require a detailed comparative analysis.

The aim. To perform a comparative analysis of clinical and functional characteristics, as well as disease course, in patients with IPF, fHP, and fILD-SSc with the UIP pattern on HRCT.

Methods. This retrospective two-center study included a total of 554 patients: 219 with IPF, 280 with fHP, and 55 with fILD-SSc. HRCT patterns were classified as typical UIP and probable UIP by radiology experts who assessed the images. We compared clinical, functional, and hemodynamic parameters, and identified factors associated with the presence of the UIP pattern as well as predictors of disease progression over a 12-month follow-up period.

Results. The typical UIP pattern was identified in 43.4% of patients with IPF, 40.7% of patients with fHP, and 16.4% of patients with fILD-SSc (p < 0.001). Independent predictors of honeycombing in non-IPF fibrotic ILDs were male sex, high index scores GAP (Gender, Age, Physiology), digital clubbing, smoking pack-years, and the presence of cough (p < 0.05 for all). Male sex (OR – 2.25; p = 0.05) and GAP index scores (OR – 1.48; p = 0.012) remained the most significant factors in the multiple regression model. The UIP pattern was a predictor of disease progression in the fHP and fILD-SSc cohort, with the OR of 2.06 (95% CI – 1.07 – 3.96, p = 0.03).

Conclusion. The UIP pattern serves as a significant prognostic marker for progression in patients with fHP and fILD-SSc. This finding underscores the necessity for enhanced monitoring and consideration of early antifibrotic therapy in these patients.

1. Ryerson C.J., Adegunsoye A., Piciucchi S. et al. Update of the International multidisciplinary classification of the interstitial pneumonias: an ERS/ATS statement. Eur. Respir. J. 2025; 4; 66 (6): 2500158. DOI: 10.1183/13993003.00158-2025.

2. Avdeev S.N., Chikina S.Y., Tyurin I.E. et al. [Chronic fibrosing interstitial lung diseases with progressive fibrotic phenotype: Resolution of the Interdisciplinary Council of Experts]. Pul'monologiya. 2021; 31 (4): 505–510. DOI: 10.18093/0869-0189-2021-31-4-505-510 (in Russian).

3. 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): e18–47. DOI: 10.1164/rccm.202202-0399ST.

4. Avdeev S.N., Aisanov Z.R., Belevskiy A.S. et al. [Idiopathic pulmonary fibrosis: federal clinical guidelines on diagnosis and treatment]. Pul'monologiya. 2022; 32 (3): 473–495. DOI: 10.18093/0869-0189-2022-32-3-473-495 (in Russian).

5. Raghu G., Remy-Jardin M., Ryerson C.J. et al. Diagnosis of hypersensitivity pneumonitis in adults. An official ATS/JRS/ALAT clinical practice guideline. Am. J. Respir. Crit. Care Med. 2020; 202 (3): e36–69. DOI: 10.1164/rccm.202005-2032ST.

6. Seixas E., Ferreira M., Serra P. et al. Criteria for progressive fibrotic hypersensitivity pneumonitis in a Portuguese patient cohort. Afr. J. Thorac. Crit. Care Med. 2022; 28 (4): e250. DOI: 10.7196/AJTCCM.2022.v28i4.250.

7. Hamblin M., Prosch H., Vašáková M. Diagnosis, course and management of hypersensitivity pneumonitis. Eur. Respir. Rev. 2022; 31 (163): 210169. DOI: 10.1183/16000617.0169-2021.

8. Trushenko N.V., Suvorova O.A., Pershina E.S. et al. Predictors of progression and mortality in patients with chronic hypersensitivity pneumonitis: retrospective analysis of registry of fibrosing interstitial lung diseases. Life (Basel). 2023; 13 (2): 467. DOI: 10.3390/life13020467.

9. Volkmann E.R., Andréasson K., Smith V. Systemic sclerosis. Lancet. 2023; 401 (10373): 304–318. DOI: 10.1016/S0140-6736(22)01692-0.

10. Elhai M., Meune C., Boubaya M. et al. Mapping and predicting mortality from systemic sclerosis. Ann. Rheum. Dis. 2017; 76 (11): 1897–1905. DOI: 10.1136/annrheumdis-2017-211448.

11. Amorim F.G., Dos Santos E.R., Yuji Verrastro C.G., Kayser C. Quantitative chest computed tomography predicts mortality in systemic sclerosis: a longitudinal study. PLoS One. 2024; 19 (9): e0310892. DOI: 10.1371/journal.pone.0310892.

12. Avdeev S.N., Aisanov Z.R., Vizel' A.A. et al. [Federal clinical guidelines on diagnosis and treatment of hypersensitivity pneumonitis]. Pul'monologiya. 2025; 35 (1): 16–41. DOI: 10.18093/0869-0189-2025-35-1-16-41 (in Russian).

13. 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. Arthritis Rheum. 2013; 65 (11): 2737–2747. DOI: 10.1002/art.38098.

14. Salisbury M.L., Tolle L.B., Xia M. et al. Possible UIP pattern on high-resolution computed tomography is associated with better survival than definite UIP in IPF patients. Respir. Med. 2017; 131: 229–235. DOI: 10.1016/j.rmed.2017.08.025.

15. Wang Y., Guo Z., Ma R. et al. Prognostic predictive characteristics in patients with fibrosing interstitial lung disease: a retrospective cohort study. Front. Pharmacol. 2022; 13: 924754. DOI: 10.3389/fphar.2022.924754.

16. Chen T., Yin C.S., Wang P. et al. Differences between patients with probable UIP and definite UIP on HRCT in idiopathic pulmonary fibrosis: a real-world cohort study. J. Clin. Med. 2024; 13 (23): 7170. DOI: 10.3390/jcm13237170.

17. Adegunsoye A., Oldham J.M., Bellam S.K. et al. Computed tomography honeycombing identifies a progressive fibrotic phenotype with increased mortality across diverse interstitial lung diseases. Ann. Am. Thorac. Soc. 2019; 16 (5): 580–588. DOI: 10.1513/AnnalsATS.201807-443OC.

18. Roeser A., Brillet P.Y., Tran Ba S. et al. The importance of considering progression speed in systemic sclerosis-associated interstitial lung diseases: application of 2022 and 2024 clinical practice guidelines for progressive pulmonary fibrosis, a retrospective cohort study. Respir. Res. 2025; 26 (1): 305. DOI: 10.1186/s12931-025-03375-7.

19. De Santis M., Bosello S.L., Peluso G. et al. Bronchoalveolar lavage fluid and progression of scleroderma interstitial lung disease. Clin. Respir. J. 2012; 6 (1): 9–17. DOI: 10.1111/j.1752-699X.2010.00228.x.

20. Gerolymatou N., Koletsos N., Karakosta M. et al. AB0838 pulmonary and cardiovascular manifestations in systemic sclerosis: results from a SSC patients cohort at a tertiary centre of nw greece. Ann. Rheum. Dis. 2023; 82: 1632. DOI: 10.1136/annrheumdis-2023-eular.5554.

21. Cai M., Zhu M., Ban C. et al. Clinical features and outcomes of 210 patients with idiopathic pulmonary fibrosis. Chin. Med. J. 2014; 127 (10): 1868–1873. DOI: 10.3760/cma.j.issn.0366-6999.20132528.

22. Myall K.J., Kavanagh J.E., Birring S.S. Idiopathic pulmonary fibrosis-associated cough: mechanisms and management. Pulm. Pharmacol. Ther. 2019; 56: 100–103. DOI: 10.1016/j.pupt.2019.03.008.

23. Froese A.R., Shimbori C., Bellaye P.S. et al. Stretch induced activation of transforming growth factor-β1 in pulmonary fibrosis. Am. J. Respir. Crit. Care Med. 2016; 194 (1): 84–96. DOI: 10.1164/rccm.201508-1638OC.

24. Theodore A.C., Tseng C.H., Li N. et al. Correlation of cough with disease activity and treatment with cyclophosphamide in scleroderma interstitial lung disease: findings from the scleroderma lung study. Chest. 2012; 142 (3): 614–621. DOI: 10.1378/chest.11-0801.

25. Tashkin D.P., Volkmann E.R., Tseng C.H. et al. Improved cough and cough-specific quality of life in patients treated for scleroderma-related interstitial lung disease: results of scleroderma lung study II. Chest. 2017; 151 (4): 813–820. DOI: 10.1016/j.chest.2016.11.052.

26. Liao Y.W., Liu M.C., Wu Y.C. et al. Factors influencing long-term outcomes in fibrotic interstitial lung disease (F-ILD) diagnosed through multidisciplinary discussion (MDD): a prospective cohort study. Eur. J. Med. Res. 2024; 29 (1): 91. DOI: 10.1186/s40001-024-01673-2.

27. Mura M., Rellini C., Taha N. et al. Radiographic progression and survival of the different HRCT patterns of idiopathic pulmonary fibrosis. Sarcoidosis Vasc. Diffuse Lung Dis. 2022; 39 (2): e2022021. DOI: 10.36141/svdld.v39i2.12534.

28. Kwon B.S., Choe J., Do K.H. et al. Computed tomography patterns predict clinical course of idiopathic pulmonary fibrosis. Respir. Res. 2020; 21 (1): 295. DOI: 10.1186/s12931-020-01562-2.

29. Gayá García-Manso I., Arenas Jiménez J., Hernández Blasco L. et al. Radiological usual interstitial pneumonia pattern is associated with two-year mortality in patients with idiopathic pulmonary fibrosis. Heliyon. 2024; 10 (5): e26623. DOI: 10.1016/j.heliyon.2024.e26623.

30. Gu J.M., Xiao S.Y., Chen B.T. et al. A clinical predictive model for the long-term survival of progressive fibrosis interstitial lung disease patients. Ther. Adv. Respir. Dis. 2025; 19: 17534666251379586. DOI: 10.1177/17534666251379586.

31. Marinescu D.C., Hague C.J., Muller N.L. et al. CT honeycombing and traction bronchiectasis extent independently predict survival across fibrotic interstitial lung disease subtypes. Radiology. 2025; 314 (2): e241001. DOI: 10.1148/radiol.241001.

32. Salisbury M.L., Gu T., Murray S. et al. Hypersensitivity pneumonitis: radiologic phenotypes are associated with distinct survival time and pulmonary function trajectory. Chest. 2019; 155 (4): 699–711. DOI: 10.1016/j.chest.2018.08.1076.

33. Resende A.C., Cordero S., Mancuzo E.V. et al. Progression in fibrotic interstitial lung diseases: prevalence and indicators in the initial evaluation in a Brazilian multicentric cohort. Cureus. 2025; 17 (3): e80290. DOI: 10.7759/cureus.80290.

34. Adams T.N., Batra K., Kypreos M., Glazer C.S. Impact of radiographic honeycombing on transplant free survival and efficacy of immunosuppression in fibrotic hypersensitivity pneumonitis. BMC Pulm. Med. 2023; 23 (1): 224. DOI: 10.1186/s12890-023-02523-3.

35. Petrarulo S., Ravaglia C., Kronborg White S. et al. The clinical meaning of the UIP pattern in fibrotic hypersensitivity pneumonitis on cryobiopsy: a multicentre retrospective study. Pulmonology. 2025; 31 (1): 242550. DOI: 10.1080/25310429.2024.2425503.

36. Hoffmann-Vold A.M., Allanore Y., Alves M. et al. Progressive interstitial lung disease in patients with systemic sclerosis-associated interstitial lung disease in the EUSTAR database. Ann. Rheum. Dis. 2021; 80 (2): 219–227. DOI: 10.1136/annrheumdis-2020-217455.

37. Durheim M.T., Hoffmann-Vold A.M., Eagan T.M. et al. ILD-specific health-related quality of life in systemic sclerosis-associated ILD compared with IPF. BMJ Open Respir. Res. 2020; 7 (1): e000598. DOI: 10.1136/bmjresp-2020-000598.