Prognostic Significance of Histopathologic Subsets in Idiopathic Pulmonary Fibrosis
Abstract
Idiopathic pulmonary fibrosis (IPF) is a generally fatal disorder with a reported median survival of 3 to 6 yr. This has been based on relatively few studies with diagnoses inconsistently confirmed by adequate lung biopsy. Retrospective analysis of 104 patients with IPF who had open lung biopsy (OLB) at Mayo Medical Center from 1976 to 1985 was performed to establish the overall survival rate, the spectrum of histopathological subgroups and their associated prognostic significance. The study group consisted of 54 men and 50 women with a median age of 63 yr. Median survival was 3.8 yr after diagnosis by OLB with an estimated 10 yr survival of 27%. Current histopathologic review showed a heterogeneous group including usual interstitial pneumonia (UIP), desquamative interstitial pneumonia (DIP), nonspecific interstitial pneumonia/fibrosis (NSIP), acute interstitial pneumonia (AIP), bronchiolitis, bronchiolitis obliterans organizing pneumonia (BOOP), and others. Median survival of the UIP group was 2.8 yr which is significantly worse (p < 0.001) than for other subgroups of chronic interstitial pneumonias. IPF includes several histopathologic subgroups with significantly different survival rates. Patients with UIP have worse survival than patients with other types of idiopathic chronic interstitial pneumonias including NSIP. Accurate histopathologic classification is essential for prognostication in patients with IPF.
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The median survival time for patients with IPF is 2 to 3 years from diagnosis and the 5-year survival rate ranges between 30% and 50%.
“The median survival time for patients with IPF is 2 to 3 years from diagnosis and the 5-year survival rate ranges between 30% and 50% [1,5-7].”
From Lung transplantation in idiopathic pulmonary fibrosis: a systematic review of the literature (2014) · cites this paper as [7]
Cites
Nonparametric Estimation from Incomplete Observations
Kaplan, E. L., & Meier, P. (1958) · Journal of the American Statistical Association · doi:10.1080/01621459.1958.10501452 · Cited by 39,158 · see citing works
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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-citation(2002) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/ajrccm.165.2.ats01 · Cited by 4,196 · see citing works
Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials
Noble, P. W., Albera, C., Bradford, W. Z., Costabel, U., Glassberg, M. K., Kardatzke, D., King, T. E., Lancaster, L., Sahn, S. A., Szwarcberg, J., et al. (2011) · The Lancet · doi:10.1016/s0140-6736(11)60405-4 · Cited by 2,154 · see citing works
Clinical course and prediction of survival in idiopathic pulmonary fibrosis
Ley, B., Collard, H. R., & King, T. E. (2011) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201006-0894ci · Cited by 1,765 · see citing works
A Multidimensional Index and Staging System for Idiopathic Pulmonary Fibrosis
Ley, B., Ryerson, C. J., Vittinghoff, E., Ryu, J. H., Tomassetti, S., Lee, J. S., Poletti, V., Buccioli, M., Elicker, B. M., Jones, K. D., et al. (2012) · Annals of Internal Medicine · doi:10.7326/0003-4819-156-10-201205150-00004 · Cited by 1,308 · see citing works
Self-citationIdiopathic Pulmonary Fibrosis: Clinical Relevance of Pathologic Classification
KATZENSTEIN, A.-L. A., & MYERS, J. L. (1998) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/ajrccm.157.4.9707039 · Cited by 1,261 · see citing works
Self-citationHigh-Dose Acetylcysteine in Idiopathic Pulmonary Fibrosis
Demedts, M., Behr, J., Buhl, R., Costabel, U., Dekhuijzen, R., Jansen, H. M., MacNee, W., Thomeer, M., Wallaert, B., Laurent, F., et al. (2005) · New England Journal of Medicine · doi:10.1056/nejmoa042976 · Cited by 964 · 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
BUILD-3: A Randomized, Controlled Trial of Bosentan in Idiopathic Pulmonary Fibrosis
King, T. E., Brown, K. K., Raghu, G., du Bois, R. M., Lynch, D. A., Martinez, F., Valeyre, D., Leconte, I., Morganti, A., Roux, S., et al. (2011) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201011-1874oc · Cited by 718 · see citing works
Wells, A. U., Desai, S. R., Rubens, M. B., Goh, N. S. L., Cramer, D., Nicholson, A. G., Colby, T. V., du Bois, R. M., & Hansell, D. M. (2003) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.2111053 · Cited by 686 · see citing works
A placebo-controlled trial of interferon gamma-1b in patients with idiopathic pulmonary fibrosis
Raghu, G., Brown, K. K., Bradford, W. Z., Starko, K., Noble, P. W., Schwartz, D. A., & King, T. E. (2004) · New England Journal of Medicine · doi:10.1056/nejmoa030511 · Cited by 652 · see citing works
A Controlled Trial of Sildenafil in Advanced Idiopathic Pulmonary Fibrosis
(2010) · New England Journal of Medicine · doi:10.1056/nejmoa1002110 · Cited by 634 · see citing works
Predicting Survival Across Chronic Interstitial Lung Disease
Ryerson, C. J., Vittinghoff, E., Ley, B., Lee, J. S., Mooney, J. J., Jones, K. D., Elicker, B. M., Wolters, P. J., Koth, L. L., King, T. E., et al. (2014) · Chest · doi:10.1378/chest.13-1474 · Cited by 558 · see citing works
King, T. E., Albera, C., Bradford, W. Z., Costabel, U., Hormel, P., Lancaster, L., Noble, P. W., Sahn, S. A., Szwarcberg, J., Thomeer, M., et al. (2009) · The Lancet · doi:10.1016/s0140-6736(09)60551-1 · Cited by 531 · see citing works
BUILD-1: A Randomized Placebo-controlled Trial of Bosentan in Idiopathic Pulmonary Fibrosis
King, T. E., Behr, J., Brown, K. K., du Bois, R. M., Lancaster, L., de Andrade, J. A., Stähler, G., Leconte, I., Roux, S., & Raghu, G. (2008) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.200705-732oc · Cited by 500 · see citing works
Telomere-related lung fibrosis is diagnostically heterogeneous but uniformly progressive
Newton, C. A., Batra, K., Torrealba, J., Kozlitina, J., Glazer, C. S., Aravena, C., Meyer, K., Raghu, G., Collard, H. R., & Garcia, C. K. (2016) · European Respiratory Journal · doi:10.1183/13993003.00308-2016 · Cited by 400 · see citing works
Stuart, 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
Idiopathic Pulmonary Fibrosis: Clinically Meaningful Primary Endpoints in Phase 3 Clinical Trials
Raghu, G., Collard, H. R., Anstrom, K. J., Flaherty, K. R., Fleming, T. R., King, T. E., Martinez, F. J., & Brown, K. K. (2012) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201201-0006pp · Cited by 242 · see citing works
DOUGLAS, W. W., RYU, J. H., SWENSEN, S. J., OFFORD, K. P., SCHROEDER, D. R., CARON, G. M., & DeREMEE, R. A. (1998) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/ajrccm.158.1.9709089 · Cited by 182 · see citing works
Self-citationMoodley, Y. P., Scaffidi, A. K., Misso, N. L., Keerthisingam, C., McAnulty, R. J., Laurent, G. J., Mutsaers, S. E., Thompson, P. J., & Knight, D. A. (2003) · The American Journal of Pathology · doi:10.1016/s0002-9440(10)63658-9 · Cited by 165 · see citing works
Lung transplantation in idiopathic pulmonary fibrosis: a systematic review of the literature
Kistler, K. D., Nalysnyk, L., Rotella, P., & Esser, D. (2014) · BMC Pulmonary Medicine · doi:10.1186/1471-2466-14-139 · Cited by 151 · see citing works
“The median survival time for patients with IPF is 2 to 3 years from diagnosis and the 5-year survival rate ranges between 30% and 50% [1,5-7].”
BackgroundCombination therapy: the future of management for idiopathic pulmonary fibrosis?
Wuyts, W. A., Antoniou, K. M., Borensztajn, K., Costabel, U., Cottin, V., Crestani, B., Grutters, J. C., Maher, T. M., Poletti, V., Richeldi, L., et al. (2014) · The Lancet Respiratory Medicine · doi:10.1016/s2213-2600(14)70232-2 · Cited by 150 · see citing works
Rudd, R. M., Prescott, R. J., Chalmers, J. C., Johnston, I. D. A., & for the Fibrosing Alveolitis Subcommittee of the Research Committee of the British Thoracic Society (2007) · Thorax · doi:10.1136/thx.2005.045591 · Cited by 113 · see citing works
Evaluating disease severity in idiopathic pulmonary fibrosis
Robbie, H., Daccord, C., Chua, F., & Devaraj, A. (2017) · European Respiratory Review · doi:10.1183/16000617.0051-2017 · Cited by 93 · see citing works
Torrisi, S. E., Ley, B., Kreuter, M., Wijsenbeek, M., Vittinghoff, E., Collard, H. R., & Vancheri, C. (2019) · European Respiratory Journal · doi:10.1183/13993003.01587-2018 · Cited by 79 · see citing works
Proteomic Biomarkers of Survival in Idiopathic Pulmonary Fibrosis
Oldham, J. M., Huang, Y., Bose, S., Ma, S.-F., Kim, J. S., Schwab, A., Ting, C., Mou, K., Lee, C. T., Adegunsoye, A., et al. (2024) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.202301-0117oc · Cited by 72 · see citing works
Hayes, D., Black, S. M., Tobias, J. D., Kirkby, S., Mansour, H. M., & Whitson, B. A. (2016) · The Annals of Thoracic Surgery · doi:10.1016/j.athoracsur.2015.06.024 · Cited by 66 · see citing works
Hayes, D., Higgins, R. S., Black, S. M., Wehr, A. M., Lehman, A. M., Kirkby, S., & Whitson, B. A. (2015) · The Journal of Heart and Lung Transplantation · doi:10.1016/j.healun.2014.09.004 · Cited by 41 · see citing works
- Authors
- BJORAKER, J. A., RYU, J. H., EDWIN, M. K., MYERS, J. L., TAZELAAR, H. D., SCHROEDER, D. R., & OFFORD, K. P.
- Affiliations
- Mayo Clinic (US)
- Venue
- American Journal of Respiratory and Critical Care Medicine
- Publisher
- Oxford University Press (OUP)
- Published
- 1998-01-01
- Volume
- 157
- Issue
- 1
- Pages
- 199-203
- ISSN
- 1073-449X (ISSN-L)
- 1535-4970
- OpenAlex
- W2140923971
- Cited by
- 1,039
- References
- 1
- Citations by year
- 2026: 10 · 2025: 8 · 2024: 16 · 2023: 16 · 2022: 22 · 2021: 25 · 2020: 24 · 2019: 33 · 2018: 39 · 2017: 38 · 2016: 39 · 2015: 48 · 2014: 48 · 2013: 53 · 2012: 44
- FWCI
- 34.57
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- American Journal of Respiratory and Critical Care Medicine (publishedVersion)
- PubMed (publishedVersion)
- 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 › Surgery › Transplantation: Methods and Outcomes
- Keywords
- idiopathic pulmonary fibrosis, prognostic value, survival, usual interstitial pneumonia, nonspecific interstitial pneumonia, desquamative interstitial pneumonia, acute interstitial pneumonia, cryptogenic organizing pneumonia, chronic interstitial pneumonia, histological classification, open lung biopsy
- MeSH terms
- Adult, Aged, Aged, 80 and over, Anti-Inflammatory Agents, Biopsy, Female, Humans, Male, Middle Aged, Predictive Value of Tests, Prednisone, Prognosis, Pulmonary Fibrosis, Retrospective Studies, Proportional Hazards Models, Survival Analysis
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