Proteomic Biomarkers of Survival in Idiopathic Pulmonary Fibrosis
Open accessAbstract
Rationale: Idiopathic pulmonary fibrosis (IPF) causes progressive lung scarring and high mortality. Reliable and accurate prognostic biomarkers are urgently needed. Objectives: To identify and validate circulating protein biomarkers of IPF survival. Methods: High-throughput proteomic data were generated using prospectively collected plasma samples from patients with IPF from the Pulmonary Fibrosis Foundation Patient Registry (discovery cohort) and the Universities of California, Davis; Chicago; and Virginia (validation cohort). Proteins associated with three-year transplant-free survival (TFS) were identified using multivariable Cox proportional hazards regression. Those associated with TFS after adjustment for false discovery in the discovery cohort were advanced for testing in the validation cohort, with proteins maintaining TFS association with consistent effect direction considered validated. After combining cohorts, functional analyses were performed, and machine learning was used to derive a proteomic signature of TFS. Measurements and Main Results: Of 2,921 proteins tested in the discovery cohort (n = 871), 231 were associated with differential TFS. Of these, 140 maintained TFS association with consistent effect direction in the validation cohort (n = 355). After cohorts were combined, the validated proteins with the strongest TFS association were latent-transforming growth factor β-binding protein 2 (hazard ratio [HR], 2.43; 95% confidence interval [CI] = 2.09–2.82), collagen α-1(XXIV) chain (HR, 2.21; 95% CI = 1.86–2.39), and keratin 19 (HR, 1.60; 95% CI = 1.47–1.74). In decision curve analysis, a proteomic signature of TFS outperformed a similarly derived clinical prediction model. Conclusions: In the largest proteomic investigation of IPF outcomes performed to date, we identified and validated 140 protein biomarkers of TFS. These results shed important light on potential drivers of IPF progression.
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Abundance at week 12 of proteins previously reported to be associated with lung function and transplant-free survival in patients with IPF, such as ADAMTSL2, COL6A3, CCN3, COL24A1, KRT19, and LTBP2, were associated with change in FVC.
“We further found that the abundance at week 12 of proteins previously reported to be associated with lung function38 and transplant-free survival39 in patients with IPF, such as ADAMTSL2, COL6A3, CCN3, COL24A1, KRT19 and LTBP2, were associated change in FVC”
From A generative AI-discovered TNIK inhibitor for idiopathic pulmonary fibrosis: a randomized phase 2a trial (2025) · cites this paper as [39]
MMP10, LTBP2, KRT19, and COL24A1 have been nominated as biomarkers for IPF and transplant-free survival.
“MMP1047, LTBP239,48,49, KRT1939 and COL24A139 have been nominated as biomarkers for IPF and transplant-free survival”
From A generative AI-discovered TNIK inhibitor for idiopathic pulmonary fibrosis: a randomized phase 2a trial (2025) · cites this paper as [39]
Cites
Szklarczyk, D., Gable, A. L., Lyon, D., Junge, A., Wyder, S., Huerta-Cepas, J., Simonovic, M., Doncheva, N. T., Morris, J. H., Bork, P., et al. (2019) · Nucleic Acids Research · doi:10.1093/nar/gky1131 · Cited by 17,749 · see citing works
Raghu, G., Collard, H. R., Egan, J. J., Martinez, F. J., Behr, J., Brown, K. K., Colby, T. V., Cordier, J.-F., Flaherty, K. R., Lasky, J. A., et al. (2011) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.2009-040gl · Cited by 7,186 · see citing works
Self-citationEfficacy and Safety of Nintedanib in Idiopathic Pulmonary Fibrosis
Richeldi, L., du Bois, R. M., Raghu, G., Azuma, A., Brown, K. K., Costabel, U., Cottin, V., Flaherty, K. R., Hansell, D. M., Inoue, Y., et al. (2014) · New England Journal of Medicine · doi:10.1056/nejmoa1402584 · Cited by 4,654 · see citing works
Self-citationDiagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline
Raghu, G., Remy-Jardin, M., Myers, J. L., Richeldi, L., Ryerson, C. J., Lederer, D. J., Behr, J., Cottin, V., Danoff, S. K., Morell, F., et al. (2018) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201807-1255st · Cited by 4,103 · see citing works
Self-citationLederer, D. J., & Martinez, F. J. (2018) · New England Journal of Medicine · doi:10.1056/nejmra1705751 · Cited by 2,056 · see citing works
Assarsson, E., Lundberg, M., Holmquist, G., Björkesten, J., Bucht Thorsen, S., Ekman, D., Eriksson, A., Rennel Dickens, E., Ohlsson, S., Edfeldt, G., et al. (2014) · PLoS ONE · doi:10.1371/journal.pone.0095192 · Cited by 1,874 · see citing works
A simple, step-by-step guide to interpreting decision curve analysis
Vickers, A. J., van Calster, B., & Steyerberg, E. W. (2019) · Diagnostic and Prognostic Research · doi:10.1186/s41512-019-0064-7 · Cited by 1,174 · see citing works
Prognostic Significance of Histopathologic Subsets in Idiopathic Pulmonary Fibrosis
BJORAKER, J. A., RYU, J. H., EDWIN, M. K., MYERS, J. L., TAZELAAR, H. D., SCHROEDER, D. R., & OFFORD, K. P. (1998) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/ajrccm.157.1.9704130 · Cited by 1,039 · see citing works
Predicting Survival in Idiopathic Pulmonary Fibrosis
KING, T. E., TOOZE, J. A., SCHWARZ, M. I., BROWN, K. R., & CHERNIACK, R. M. (2001) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/ajrccm.164.7.2003140 · Cited by 853 · see citing works
Raghu, G., Chen, S.-Y., Yeh, W.-S., Maroni, B., Li, Q., Lee, Y.-C., & Collard, H. R. (2014) · The Lancet Respiratory Medicine · doi:10.1016/s2213-2600(14)70101-8 · Cited by 716 · see citing works
MMP1 and MMP7 as Potential Peripheral Blood Biomarkers in Idiopathic Pulmonary Fibrosis
Rosas, I. O., Richards, T. J., Konishi, K., Zhang, Y., Gibson, K., Lokshin, A. E., Lindell, K. O., Cisneros, J., MacDonald, S. D., Pardo, A., et al. (2008) · PLoS Medicine · doi:10.1371/journal.pmed.0050093 · Cited by 582 · see citing works
Peripheral blood proteins predict mortality in idiopathic pulmonary fibrosis
Richards, T. J., Kaminski, N., Baribaud, F., Flavin, S., Brodmerkel, C., Horowitz, D., Li, K., Choi, J., Vuga, L. J., Lindell, K. O., et al. (2012) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201101-0058oc · Cited by 381 · see citing works
Serum CC-Chemokine Ligand 18 Concentration Predicts Outcome in Idiopathic Pulmonary Fibrosis
Prasse, A., Probst, C., Bargagli, E., Zissel, G., Toews, G. B., Flaherty, K. R., Olschewski, M., Rottoli, P., & Müller–Quernheim, J. (2009) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.200808-1201oc · Cited by 329 · see citing works
Self-citationHerazo-Maya, J. D., Noth, I., Duncan, S. R., Kim, S., Ma, S.-F., Tseng, G. C., Feingold, E., Juan-Guardela, B. M., Richards, T. J., Lussier, Y., et al. (2013) · Science Translational Medicine · doi:10.1126/scitranslmed.3005964 · Cited by 325 · see citing works
Self-citationStuart, B. D., Lee, J. S., Kozlitina, J., Noth, I., Devine, M. S., Glazer, C. S., Torres, F., Kaza, V., Girod, C. E., Jones, K. D., et al. (2014) · The Lancet Respiratory Medicine · doi:10.1016/s2213-2600(14)70124-9 · Cited by 310 · see citing works
Self-citationJacob, J., Bartholmai, B. J., Rajagopalan, S., Kokosi, M., Nair, A., Karwoski, R., Walsh, S. L. F., Wells, A. U., & Hansell, D. M. (2017) · European Respiratory Journal · doi:10.1183/13993003.01011-2016 · Cited by 309 · see citing works
Maher, T. M., Oballa, E., Simpson, J. K., Porte, J., Habgood, A., Fahy, W. A., Flynn, A., Molyneaux, P. L., Braybrooke, R., Divyateja, H., et al. (2017) · The Lancet Respiratory Medicine · doi:10.1016/s2213-2600(17)30430-7 · Cited by 271 · see citing works
Serum Surfactant Protein-A Is a Strong Predictor of Early Mortality in Idiopathic Pulmonary Fibrosis
Kinder, B. W., Brown, K. K., McCormack, F. X., Ix, J. H., Kervitsky, A., Schwarz, M. I., & King, T. E. (2009) · CHEST Journal · doi:10.1378/chest.08-2209 · Cited by 208 · see citing works
C-X-C Motif Chemokine 13 (CXCL13) is a Prognostic Biomarker of Idiopathic Pulmonary Fibrosis
Vuga, L. J., Tedrow, J. R., Pandit, K. V., Tan, J., Kass, D. J., Xue, J., Chandra, D., Leader, J. K., Gibson, K. F., Kaminski, N., et al. (2014) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201309-1592oc · Cited by 197 · see citing works
Herazo-Maya, J. D., Sun, J., Molyneaux, P. L., Li, Q., Villalba, J. A., Tzouvelekis, A., Lynn, H., Juan-Guardela, B. M., Risquez, C., Osorio, J. C., et al. (2017) · The Lancet Respiratory Medicine · doi:10.1016/s2213-2600(17)30349-1 · Cited by 175 · see citing works
Self-citationNewton, C. A., Oldham, J. M., Ley, B., Anand, V. C., Adegunsoye, A., Liu, G. Y., Batra, K., Torrealba, J. R., Kozlitina, J., Glazer, C. S., et al. (2019) · European Respiratory Journal · doi:10.1183/13993003.01641-2018 · Cited by 173 · see citing works
Self-citationClinical Effectiveness of Antifibrotic Medications for Idiopathic Pulmonary Fibrosis
Dempsey, T. M., Sangaralingham, L. R., Yao, X., Sanghavi, D. M., Shah, N. D., & LIMPER, A. H. (2019) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201902-0456oc · Cited by 159 · see citing works
Surfactant Protein A Predicts Survival in Idiopathic Pulmonary Fibrosis
McCormack, F. X., King, T. E., Bucher, B. L., Nielsen, L., Mason, R. J., & McCormac, F. X. (1995) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/ajrccm.152.2.7633738 · Cited by 125 · see citing works
Serum and BALF YKL-40 levels are predictors of survival in idiopathic pulmonary fibrosis
Korthagen, N. M., van Moorsel, C. H. M., Barlo, N. P., Ruven, H. J. T., Kruit, A., Heron, M., van den Bosch, J. M. M., & Grutters, J. C. (2010) · Respiratory Medicine · doi:10.1016/j.rmed.2010.09.012 · Cited by 118 · see citing works
Bowman, W. S., Newton, C. A., Linderholm, A., Neely, M. L., Pugashetti, J. V., Kaul, B., Vo, V., Echt, G., Leon, W., Shah, R., et al. (2022) · The Lancet Respiratory Medicine · doi:10.1016/s2213-2600(21)00503-8 · Cited by 111 · see citing works
Self-citationαvβ6 integrin may be a potential prognostic biomarker in interstitial lung disease
Saini, G., Porte, J., Weinreb, P. H., Violette, S. M., Wallace, W. A., McKeever, T. M., & Jenkins, G. (2015) · European Respiratory Journal · doi:10.1183/09031936.00210414 · Cited by 106 · see citing works
Parimon, T., Yao, C., Habiel, D. M., Ge, L., Bora, S. A., Brauer, R., Evans, C. M., Xie, T., Alonso-Valenteen, F., Medina‐Kauwe, L. K., et al. (2019) · JCI Insight · doi:10.1172/jci.insight.129359 · Cited by 105 · see citing works
Dual inhibition of αvβ6 and αvβ1 reduces fibrogenesis in lung tissue explants from patients with IPF
Decaris, M. L., Schaub, J. R., Chen, C., Cha, J., Lee, G. G., Rexhepaj, M., Ho, S. S., Rao, V., Marlow, M. M., Kotak, P., et al. (2021) · Respiratory Research · doi:10.1186/s12931-021-01863-0 · Cited by 94 · see citing works
Self-citationBiomarker signatures for progressive idiopathic pulmonary fibrosis
Clynick, B., Corte, T. J., Jo, H. E., Stewart, I., Glaspole, I. N., Grainge, C., Maher, T. M., Navaratnam, V., Hubbard, R., Hopkins, P. M. A., et al. (2022) · European Respiratory Journal · doi:10.1183/13993003.01181-2021 · Cited by 86 · see citing works
Adegunsoye, A., Alqalyoobi, S., Linderholm, A., Bowman, W. S., Lee, C. T., Pugashetti, J. V., Sarma, N., Ma, S.-F., Haczku, A., Sperling, A., et al. (2020) · Chest · doi:10.1016/j.chest.2020.04.066 · Cited by 82 · see citing works
Self-citationThe Pulmonary Fibrosis Foundation Patient Registry. Rationale, Design, and Methods
Wang, B. R., Edwards, R., Freiheit, E., Ma, Y., Burg, C., de Andrade, J. A., LANCASTER, L. H., Lindell, K. O., Nathan, S. D., Raghu, G., et al. (2020) · Annals of the American Thoracic Society · doi:10.1513/annalsats.202001-035sd · Cited by 79 · see citing works
Self-citationBowman, W. S., Echt, G., & Oldham, J. M. (2021) · Frontiers in Medicine · doi:10.3389/fmed.2021.680997 · Cited by 76 · see citing works
Self-citationHepatocyte Growth Factor and Lung Fibrosis
Crestani, B., Marchand-Adam, S., Quesnel, C., Plantier, L., Borensztajn, K., Marchal-Sommé, J., Mailleux, A., Soler, P., & Dehoux, M. (2012) · Proceedings of the American Thoracic Society · doi:10.1513/pats.201202-018aw · Cited by 75 · see citing works
Quantitative computed tomography predicts outcomes in idiopathic pulmonary fibrosis
Humphries, S. M., Mackintosh, J. A., Jo, H. E., Walsh, S. L. F., Silva, M., Calandriello, L., Chapman, S. A., Ellis, S. J., Glaspole, I., Goh, N. S. L., et al. (2022) · Respirology · doi:10.1111/resp.14333 · Cited by 74 · see citing works
Bone Marrow CD11c+ Cell–Derived Amphiregulin Promotes Pulmonary Fibrosis
Ding, L., Liu, T., Wu, Z. B., Hu, B., Nakashima, T., Ullenbruch, M. R., Santos, F. G. D. L., & Phan, S. H. (2016) · The Journal of Immunology · doi:10.4049/jimmunol.1502479 · Cited by 70 · see citing works
Wu, A. C., Kiley, J. P., Noel, P. J., Amur, S., Burchard, E. G., Clancy, J. P., Galanter, J., Inada, M., Jones, T. K., Kropski, J. A., et al. (2018) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201810-1895st · Cited by 68 · see citing works
Redente, E. F., Chakraborty, S., Sajuthi, S. P., Black, B. P., Edelman, B., Seibold, M. A., & Riches, D. W. H. (2020) · JCI Insight · doi:10.1172/jci.insight.141618 · Cited by 66 · see citing works
Lukey, P. T., Coello, C. S., Gunn, R. N., Parker, C. A., Wilson, F. J., Saleem, A., Garman, N., Costa, M. J., Kendrick, S. F. W., Onega, M., et al. (2019) · European Journal of Nuclear Medicine and Molecular Imaging · doi:10.1007/s00259-019-04586-z · Cited by 64 · see citing works
Lung Transplant in Idiopathic Pulmonary Fibrosis
George, T. J. (2011) · Archives of Surgery · doi:10.1001/archsurg.2011.239 · Cited by 62 · see citing works
Defect of Pro-Hepatocyte Growth Factor Activation by Fibroblasts in Idiopathic Pulmonary Fibrosis
Marchand-Adam, S., Fabre, A., Mailleux, A., Marchal-Sommé, J., Quesnel, C., Kataoka, H., Aubier, M., Dehoux, M., Soler, P., & Crestani, B. (2006) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.200507-1074oc · Cited by 61 · see citing works
Moving beyond AUC: decision curve analysis for quantifying net benefit of risk prediction models
Sadatsafavi, M., Adibi, A., Puhan, M. A., Gershon, A. S., Aaron, S. D., & Sin, D. D. (2021) · European Respiratory Journal · doi:10.1183/13993003.01186-2021 · Cited by 60 · see citing works
Bennett, D., Fossi, A., Bargagli, E., Refini, R. M., Pieroni, M., Luzzi, L., Ghiribelli, C., Paladini, P., Voltolini, L., & Rottoli, P. (2015) · Lung · doi:10.1007/s00408-015-9767-x · Cited by 58 · see citing works
Khan, F., Stewart, I. D., Saini, G., Robinson, K. A., & Jenkins, G. R. (2021) · European Respiratory Journal · doi:10.1183/13993003.01612-2021 · Cited by 57 · see citing works
Molyneaux, P. L., Fahy, W. A., Byrne, A. J., Braybrooke, R., Saunders, P. T., Toshner, R., Albers, G. J., Chua, F., Renzoni, E. A., Wells, A. U., et al. (2022) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.202107-1769oc · Cited by 52 · see citing works
The Proteomic Profile of Interstitial Lung Abnormalities
Axelsson, G. T., Guðmundsson, G., Pratte, K., Aspelund, T., Putman, R. K., Sanders, J. L., Gudmundsson, E. F., Hatabu, H., Guðmundsdóttir, V., Guðjónsson, A., et al. (2022) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.202110-2296oc · Cited by 52 · see citing works
Im, J., Kim, K., Hergert, P. J., & Nho, R. S. (2016) · The Journal of Pathology · doi:10.1002/path.4749 · Cited by 52 · see citing works
Ikeda, K., Shiratori, M., Chiba, H., Nishikiori, H., Yokoo, K., Saito, A., Hasegawa, Y., Kuronuma, K., Otsuka, M., Yamada, G., et al. (2017) · Respiratory Medicine · doi:10.1016/j.rmed.2017.08.021 · Cited by 32 · see citing works
Wu, N., Yu, Y., Chuang, C.-C., Wang, R., Benjamin, N. N., & Coultas, D. (2014) · Journal of Medical Economics · doi:10.3111/13696998.2014.991789 · Cited by 32 · see citing works
Antifibrotics and Reduced Mortality in Idiopathic Pulmonary Fibrosis: Immortal Time Bias
Suissa, S., & Suissa, K. (2022) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.202207-1301le · Cited by 31 · see citing works
Zou, M., Zou, J., Hu, X., Zheng, W., Zhang, M., & Cheng, Z. (2021) · Frontiers in Pharmacology · doi:10.3389/fphar.2021.788714 · Cited by 29 · see citing works
Riddell, P., Kleinerová, J., Eaton, D. A., Healy, D. G., Javadpour, H., McCarthy, J. F., Nölke, L., Redmond, K., & Egan, J. J. (2020) · European Respiratory Journal · doi:10.1183/13993003.02413-2019 · Cited by 18 · see citing works
Todd, J. L., Neely, M. L., Overton, R., Mulder, H., Roman, J., Lasky, J. A., de Andrade, J. A., Gulati, M., Huang, H., Leonard, T. B., et al. (2022) · Lung · doi:10.1007/s00408-021-00505-y · Cited by 14 · see citing works
Self-citationDifferences in Patient Outcomes across the Pulmonary Fibrosis Foundation Care Center Network
Boente, R., White, E. N., Baxter, C. M., Shore, J. E., Collard, H. R., & Lee, J. S. (2022) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.202206-1173le · Cited by 6 · see citing works
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Lancaster, L., Cottin, V., Ramaswamy, M., Wuyts, W. A., Jenkins, R. G., Scholand, M. B., Kreuter, M., Valenzuela, C., Ryerson, C. J., Goldin, J., et al. (2024) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.202403-0636oc · Cited by 80 · see citing works
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Huang, Y., Ma, S., Oldham, J. M., Adegunsoye, A., Zhu, D., Murray, S., Kim, J. S., Bonham, C. A., Strickland, E., Linderholm, A., et al. (2024) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.202309-1692oc · Cited by 46 · see citing works
Self-citationDecoding organ fibrosis: mechanistic insights and emerging therapeutic strategies
Chen, X., Zhang, J., Guo, L., Wu, C., Zhou, J., Xu, M., Mo, L., Li, Y., & He, J. (2026) · Signal Transduction and Targeted Therapy · doi:10.1038/s41392-025-02532-0 · Cited by 37 · see citing works
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Ganzleben, I., & Medoff, B. D. (2025) · iScience · doi:10.1016/j.isci.2025.113993 · Cited by 27 · see citing works
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Enomoto, T., Shirai, Y., Takeda, Y., Edahiro, R., Shichino, S., Nakayama, M., Takahashi-Itoh, M., Noda, Y., Adachi, Y., Kawasaki, T., et al. (2024) · JCI Insight · doi:10.1172/jci.insight.177937 · Cited by 20 · see citing works
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Spagnolo, P., & Maher, T. M. (2024) · Current Opinion in Pulmonary Medicine · doi:10.1097/mcp.0000000000001099 · Cited by 17 · see citing works
Mooney, J. J., Jacobs, S. S., Lefebvre, E. A., Cosgrove, G. P., Clark, A., Turner, S., Decaris, M. L., Barnes, C. N., Jurek, M., Williams, B., et al. (2024) · Annals of the American Thoracic Society · doi:10.1513/annalsats.202409-969oc · Cited by 17 · see citing works
Biological Age, Chronological Age, and Survival in Pulmonary Fibrosis: A Causal Mediation Analysis
Pugashetti, J. V., Kim, J. S., Bose, S., Adegunsoye, A., Linderholm, A., Chen, C., Strek, M. E., Flaherty, K. R., Murray, S., Newton, C. A., et al. (2024) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.202310-1887oc · Cited by 15 · see citing works
Self-citationMaddali, M. V., Moore, A. R., Sinha, P., Newton, C. A., Kim, J. S., Adegunsoye, A., Ma, S., Strek, M. E., Chen, C., Linderholm, A., et al. (2024) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.202402-0339oc · Cited by 14 · see citing works
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Selman, M., & Pardo, A. (2024) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.202309-1573pp · Cited by 12 · see citing works
Zhou, Y., Tong, Z., Zhu, X., Wu, C., Zhou, Y., & Dong, Z. (2025) · Journal of Translational Medicine · doi:10.1186/s12967-024-06031-8 · Cited by 12 · see citing works
Bell, J. A., Davies, E. R., Brereton, C. J., Vukmirovic, M., Roberts, J., Lunn, K., Wickens, L., Conforti, F., Ridley, R. A., Ceccato, J., et al. (2024) · Cell Reports Medicine · doi:10.1016/j.xcrm.2024.101695 · Cited by 9 · see citing works
Kim, J. S., Debban, C. L., Guzman, D. E., Hannan, R. T., Salvatore, M. M., McGroder, C. F., Zhang, D., Podolanczuk, A. J., Duprez, D. A., Ma, S., et al. (2025) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.202503-0610oc · Cited by 7 · see citing works
Self-citationWu, L., Liu, Y., Zhang, Y., Xu, R., Bi, K., Li, J., Wang, J., Liu, Y., Guo, W., Wang, Q., et al. (2024) · Scientific Reports · doi:10.1038/s41598-024-76263-7 · Cited by 7 · see citing works
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Maher, T. M., Jenkins, R. G., Cottin, V., Nishioka, Y., Noth, I., Selman, M., Song, J. W., Ittrich, C., Diefenbach, C., Stowasser, S., et al. (2024) · ERJ Open Research · doi:10.1183/23120541.00335-2023 · Cited by 7 · see citing works
Self-citationSecreted Phosphoprotein 1 in Lung Diseases
Liu, H., Coarfa, C., Charania, A. N., Larson‐Casey, J. L., Rosas, I. O., & He, C. (2025) · Metabolites · doi:10.3390/metabo15060365 · Cited by 8 · see citing works
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Paul, P., Dasgupta, S., Roychowdhury, S., Unnithan, S., Dasgupta, H., Sen, S., Madan, K., & Chaudhury, K. (2025) · Respiratory Medicine · doi:10.1016/j.rmed.2025.108487 · Cited by 6 · see citing works
Predicting and Treating Pulmonary Fibrosis with Proteomic Biomarker Investigations
Raineri, G., Samarelli, A. V., Tonelli, R., Masciale, V., Aramini, B., Petrachi, T., Bruzzi, G., Gozzi, F., Trasforini, E., Esposito, A., et al. (2025) · Biomedicines · doi:10.3390/biomedicines13112656 · Cited by 4 · see citing works
Zhavoronkov, A. A., Galkin, F., Chen, S., Ren, F., Aliper, A., Durymanov, M. O., Sidorenko, D., Cui, H., Han, J. J., Xu, H., et al. (2026) · Nature Biotechnology · doi:10.1038/s41587-026-03286-y · Cited by 4 · see citing works
Pugashetti, J. V., Kim, J. S., Combs, M. P., Ma, S., Adegunsoye, A., Linderholm, A., Strek, M. E., Chen, C., Dilling, D. F., Whelan, T. P. M., et al. (2024) · The Journal of Heart and Lung Transplantation · doi:10.1016/j.healun.2024.03.018 · Cited by 4 · see citing works
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Frantzi, M., Keller, F., Latosińska, A., Beige, J., Mebazaa, A., Caillard, A., An, D., Perco, P., Schanstra, J. P., Catanese, L., et al. (2026) · PROTEOMICS · doi:10.1002/pmic.70131 · Cited by 4 · see citing works
Weng, S., Zuo, J., Mo, J., & Ye, L. (2025) · Frontiers in Immunology · doi:10.3389/fimmu.2025.1557848 · Cited by 4 · see citing works
Circulating biomarkers in subjects with progressive pulmonary fibrosis: data from the INBUILD trial
Maher, T. M., Jenkins, G. R., Bonella, F., Assassi, S., Diefenbach, C., Ittrich, C., Rohr, K. B., & Kolb, M. (2025) · ERJ Open Research · doi:10.1183/23120541.00158-2025 · Cited by 4 · see citing works
Zhao, C., Xiao, A., Chen, C., Mu, W., Li, W.-Y., & Chang, L. (2025) · Nano Today · doi:10.1016/j.nantod.2025.102889 · Cited by 3 · see citing works
Self-citationGazing into the Proteomic Crystal Ball: Predicting Survival in Idiopathic Pulmonary Fibrosis
Lee, J. S., & Maher, T. M. (2023) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.202311-2108ed · Cited by 2 · see citing works
Enomoto, T., Takeda, Y., Shirai, Y., Hasegawa, T., Zhao, F., Lunding, H. B., Pohl, M., Edahiro, R., Shichino, S., Kawasaki, T., et al. (2025) · Respiratory Research · doi:10.1186/s12931-025-03237-2 · Cited by 2 · see citing works
Pulmonary Fibrosis—Focusing on the Future: Aspen Lung Conference 2024 Summary
Freeberg, M. A. T., Redente, E. F., Perelas, A., Riches, D. W. H., Schwartz, D. A., & Sime, P. J. (2025) · American Journal of Respiratory Cell and Molecular Biology · doi:10.1165/rcmb.2025-0137tr · Cited by 2 · see citing works
Miceli, V., Callari, A., Calzolari, E., Castelbuono, S., Claudia, C., Lanzarone, N., Martino, L., Conaldi, P. G., Pinzani, M., & Vitulo, P. (2025) · Scientific Reports · doi:10.1038/s41598-025-12952-1 · Cited by 2 · see citing works
Zhou, R., Li, G., Zhong, Q., Li, X., Liu, Y.-X., Zheng, J., Huang, H., & Wu, X. (2025) · Respiratory Research · doi:10.1186/s12931-025-03418-z · Cited by 2 · see citing works
Maddali, M. V., Pugashetti, J. V., Pienkos, S. M., Moore, A. R., Konkol, S., Kim, J. S., Ma, S.-F., Newton, C. A., Garcia, C. K., Adegunsoye, A., et al. (2026) · American Journal of Respiratory and Critical Care Medicine · doi:10.1093/ajrccm/aamag006 · Cited by 2 · see citing works
Self-citationMapping the Proteomic Landscape of Radiological Lung Abnormalities
Maddali, M. V., Kim, J. S., & Oldham, J. M. (2024) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.202402-0310ed · Cited by 2 · see citing works
Self-citationO’Dwyer, D. N., Noth, I., & Oldham, J. M. (2024) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.202312-2262le · Cited by 2 · see citing works
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Ahn, S., Fu, W., van Gerwen, M. A. G., Liu, L., & Li, Z. (2026) · PLoS Computational Biology · doi:10.1371/journal.pcbi.1014217 · Cited by 1 · see citing works
Maddali, M. V., Oldham, J. M., & Moor, C. C. (2025) · Current Opinion in Pulmonary Medicine · doi:10.1097/mcp.0000000000001199 · Cited by 1 · see citing works
Self-citationKaul, B., Petersen, L. A., Collard, H. R., Richardson, P., Zhang, N., Whooley, M. A., & Bhattacharya, M. (2025) · Annals of the American Thoracic Society · doi:10.1513/annalsats.202410-1108oc · Cited by 1 · see citing works
T Regulatory Mechanisms in Airway and Interstitial Lung Disease
Pastore, C., Stadler, B. D., Sperling, A. I., & Velez, T. E. (2025) · Seminars in Respiratory and Critical Care Medicine · doi:10.1055/a-2703-4491 · Cited by 1 · see citing works
Menon, A. A., Gansen, B. O., Mulder, H. S., Neely, M. L., Papavasileiou, P., Salisbury, M. L., Southern, B. D., Heßlinger, C., Leonard, T. B., Meissner, F., et al. (2025) · Respiratory Research · doi:10.1186/s12931-025-03377-5 · Cited by 1 · see citing works
Wang, D., Jing, Y., Zang, D., Zhang, C., & Yu, X. (2025) · Letters in Drug Design & Discovery · doi:10.1016/j.lddd.2025.100194 · Cited by 1 · see citing works
Wu, C.-S., Lai, D.-W., Chen, Y.-C., Yu, Y., Yang, L., & Fu, P.-K. (2026) · Respiratory Research · doi:10.1186/s12931-026-03596-4 · Cited by 1 · see citing works
Ruenwilai, P., Tajarernmuang, P., Chattipakorn, S. C., Chattipakorn, N., & Shinlapawittayatorn, K. (2026) · Lung · doi:10.1007/s00408-026-00887-x · Cited by 1 · see citing works
Kim, D. Y., Kim, M.-J., Oh, Y. J., Hwang, H. J., Chae, E. J., Lee, S. M., Park, S., Lee, J. C., Seo, J. B., Lee, H. Y., et al. (2026) · American Journal of Respiratory and Critical Care Medicine · doi:10.1093/ajrccm/aamag245 · Cited by 1 · see citing works
Clustering-aided prediction of outcomes in patients with idiopathic pulmonary fibrosis
Wang, L., Wu, P., Liu, Y., Patel, D. C., Leonard, T. B., & Zhao, H. (2024) · Respiratory Research · doi:10.1186/s12931-024-03015-6 · Cited by 1 · see citing works
Bodmer, N. K., Choudhury, M., Mirza, H., Pradhan, S., Yin, Y., Mecham, R. P., Brody, S. L., Ornitz, D. M., & Koenitzer, J. R. (2026) · American Journal of Respiratory Cell and Molecular Biology · doi:10.1093/ajrcmb/aanag110 · Cited by 1 · see citing works
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Menon, A. A., & Ghosh, A. (2025) · Current Opinion in Pulmonary Medicine · doi:10.1097/mcp.0000000000001192 · Cited by 1 · see citing works
Unveiling Biological Age: A New Frontier in Predicting Outcomes in Chronic Lung Disease
de Andrade, J. A., Garcia, P. A. A., & Mora, A. L. (2024) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.202407-1290ed · Cited by 1 · see citing works
Editorial: Cellular and molecular mechanisms of lung regeneration, repair, and fibrosis, volume II
Qiu, Y., Tang, X. X., Shichino, S., Kushwaha, P. P., Sanders, Y. Y., Russo, R. C., & Mo, C. (2025) · Frontiers in Cell and Developmental Biology · doi:10.3389/fcell.2025.1653034 · Cited by 1 · see citing works
Fujimoto, H., D’Alessandro‐Gabazza, C. N., Yasuma, T., Gabazza, E. C., Hataji, O., & Kobayashi, T. (2024) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.202310-1899le
Biomarker-defined endotypes of pulmonary fibrosis
McCall, A. S., & Kropski, J. A. (2024) · The Lancet Respiratory Medicine · doi:10.1016/s2213-2600(24)00169-3
Todd, J. L., Page, C. B., Wu, P., Belperio, J. A., Maher, T. M., Palmer, S. M., Leonard, T. B., Heßlinger, C., Neely, M. L., & Schlange, T. (2025) · ERJ Open Research · doi:10.1183/23120541.00738-2024
Genetic Biomarkers for Interstitial Lung Disease
Zhang, D., & Newton, C. A. (2025) · Respiratory medicine · doi:10.1007/978-3-031-87183-2_3
Big Data: Pearls and Pitfalls for Precision Medicine in Interstitial Lung Disease
Kaul, B., Collard, H. R., & Petersen, L. A. (2025) · Respiratory medicine · doi:10.1007/978-3-031-87183-2_10
Nagel, D. J., & Valenzi, E. (2025) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.202508-2007ed
Plasma proteomic biomarkers underlying polygenic risk of idiopathic pulmonary fibrosis
Zhu, J., Zhou, D., Chen, Y., You, Z., & Li, Y. (2025) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.202505-1202rl
Prioritizing Therapeutic Targets for Interstitial Lung Disease: A Causal Mediation AnalysisPreprint
Oldham, J., Molyneaux, P. L., Maddali, M. V., Newton, C. A., Kim, J. S., Konkol, S., Pugashetti, J. V., Liu, G. Y., Goobie, G. C., Adegunsoye, A., et al. (2026) · Research Square · doi:10.21203/rs.3.rs-8714555/v1
Self-citationBreath as a biomarker: harnessing volatile clues in interstitial lung disease diagnosis
Berger, K., & Aronson, K. I. (2026) · American Journal of Respiratory and Critical Care Medicine · doi:10.1093/ajrccm/aamag111
Swaminathan, A. C., Mulder, H., Neely, M. L., Schmid, R., Belperio, J. A., Patel, N. M., Palmer, S., & Todd, J. L. (2026) · Respiratory Research · doi:10.1186/s12931-026-03641-2
Challenging the status quo for therapeutic clinical trials in fibrotic interstitial lung diseases
Kulkarni, T., Funke-Chambour, M., Jones, S., Rivera‐Ortega, P., & Montesi, S. B. (2026) · Thorax · doi:10.1136/thorax-2024-222057
Treatable traits in interstitial lung disease: towards precision medicine
Avitzur, N., Marinescu, D.-C., Churg, A., Goobie, G. C., Hague, C. J., Müller, N. L., Wright, J. L., & Ryerson, C. J. (2026) · Thorax · doi:10.1136/thorax-2024-222058
Steinmetz, L.-K., Seeliger, B., Schupp, J. C., Bormann, T., Aschenbrenner, F., Maus, R., DeLuca, D. S., Gaedcke, S., Pich, A., Knudsen, L., et al. (2026) · ERJ Open Research · doi:10.1183/23120541.01320-2025
Zhou, Q., & Shen, Y. (2026) · Clinical Proteomics · doi:10.1186/s12014-026-09583-7
Drug repurposing for lung disease: mechanistic rationale, clinical evidence, and the path forward
Bhatt, S., Gyimah, C., Kaul, B., Johannson, K. A., LIMPER, A. H., & Dempsey, T. (2026) · American Journal of Respiratory and Critical Care Medicine · doi:10.1093/ajrccm/aamag216
Early Pulmonary Fibrosis is Defined by Niche- and Cell-Specific Molecular ProgramsPreprint
Waich, A., Ochsner, S. A., Villalba, J. A., Rose, J. A., Cala-García, J., Zuluaga, J. D., McKenna, N. J., Echartea, M. E. R., He, C., Celada, L. J., et al. (2026) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.64898/2026.05.28.727955
Immunosenescence in Idiopathic Pulmonary Fibrosis
Xiao, S., Li, Z., Zhang, H., Cai, W., Yu, S., & Song, X. (2026) · Journal of Inflammation Research · doi:10.2147/jir.s586167
Opportunities for human lung tissue-based studies in fibrotic interstitial lung disease research
Sturek, J. M., Kim, J. S., Podolanczuk, A. J., Hannan, R. T., LIMPER, A. H., Hariri, L. P., Richeldi, L., Rosas, I. O., Jenkins, G. R., Luo, F., et al. (2026) · American Journal of Respiratory and Critical Care Medicine · doi:10.1093/ajrccm/aamag346
Self-citationZhu, Y., Ke, Z., Zhang, T., Sun, S. X., & Fan, X. Q. (2026) · Journal of Clinical Medicine · doi:10.3390/jcm15135124
Kim, M. K., Kim, S. H., Park, S. W., & Lee, J.-U. (2026) · OMICS A Journal of Integrative Biology · doi:10.1177/15578100261467222
AI-based strategies for diagnosis and therapeutic discovery in pulmonary fibrosis
Lin, Y., Liu, Q., Lei, X.-Y., Cao, H.-Y., Feng, P.-M., Wang, Y.-M., Sun, D., Xin, G., Zhang, S., & Chen, W. (2026) · Chinese Herbal Medicines · doi:10.1016/j.chmed.2026.08.005
Markers of microvascular instability predict severity and survival in idiopathic pulmonary fibrosis
Csordas, D. J., Ma, S., Huang, Y., Receveur, B. A., Strickland, E., Cucuzzella, L. C., Pugashetti, J. V., Lee, C. T., Podolanczuk, A. J., Martínez, F. J., et al. (2026) · Thorax · doi:10.1136/thorax-2026-225147
Self-citation
- Authors
- Oldham, J. M., Huang, Y., Bose, S., Ma, S.-F., Kim, J. S., Schwab, A., Ting, C., Mou, K., Lee, C. T., Adegunsoye, A., Ghodrati, S., Pugashetti, J. V., Nazemi, N., Strek, M. E., Linderholm, A. L., Chen, C.-H., Murray, S., Zemans, R. L., Flaherty, K. R., Martinez, F. J., & Noth, I.
- Affiliations
- University of Michigan (US)
- University of Virginia (US)
- University of Chicago (US)
- University of California, Davis (US)
- Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine
- Weill Cornell Medical Center, New York, New York
- ORCID
- Venue
- American Journal of Respiratory and Critical Care Medicine
- Publisher
- Oxford University Press (OUP)
- Published
- 2024-05-01
- Volume
- 209
- Issue
- 9
- Pages
- 1111-1120
- ISSN
- 1073-449X (ISSN-L)
- 1535-4970
- PMC
- PMC11092951
- OpenAlex
- W4387701231
- Cited by
- 72
- References
- 53
- Citations by year
- 2026: 24 · 2025: 28 · 2024: 19 · 2023: 1
- FWCI
- 13.20
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- American Journal of Respiratory and Critical Care Medicine (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Heart, Lung, and Blood Institute (K23HL150301, K23HL146942, T32HL007605, T32HL007749, R35HL160770, RO1HL130796, UG3HL145266)
- Topics
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Occupational and environmental lung diseases
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Chronic Obstructive Pulmonary Disease (COPD) Research
- Keywords
- idiopathic pulmonary fibrosis, prognostic factors, survival, plasma proteomics, circulating proteins, transplant-free survival, LTBP2, keratin 19, proteomic signatures, disease progression
- MeSH terms
- Aged, Female, Humans, Male, Middle Aged, Prognosis, Prospective Studies, Cohort Studies, Biomarkers, Proportional Hazards Models, Proteomics, Idiopathic Pulmonary Fibrosis
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