Research & Literature
Explore the leading researchers and institutions driving advances in this area, and dive into the full body of literature that informs this resource.
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Royal Brompton Hospital
London, United Kingdom
Université Claude Bernard Lyon 1
Villeurbanne, France
Ulsan College
Ulsan, South Korea
Royal Brompton & Harefield NHS Foundation Trust
London, United Kingdom
Mayo Clinic
Rochester, United States
University College London
London, United Kingdom
Guy's and St Thomas' NHS Foundation Trust
London, United Kingdom
The University of Sydney
Sydney, Australia
Kanagawa Cardiovascular and Respiratory Center
Yokohama, Japan
WinnMed
Decorah, United States
References
References (63)
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Respiratory medicine 2016; (117()):14-26.
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Combined pulmonary fibrosis and emphysema: a retrospective analysis of clinical characteristics, treatment and prognosis.
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[THE SYNDROME OF COMBINED PULMONARY FIBROSIS AND EMPHYSEMA - CPFE].
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Presence of lung cancer and high gender, age, and physiology score as predictors of acute exacerbation in combined pulmonary fibrosis and emphysema: A retrospective study.
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Combined pulmonary fibrosis and emphysema in systemic sclerosis: A syndrome associated with heavy morbidity and mortality.
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Seminars in arthritis and rheumatism 2019; (49(1)):98-104 doi:10.1016/j.semarthrit.2018.10.011.
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HRCT diagnosis of combined pulmonary fibrosis and emphysema in a patient of chronic obstructive pulmonary disease with pulmonary hypertension and clinical or radiograph suspicion of pulmonary fibrosis.
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BJR case reports 2016; (2(4)):20150070 doi:10.1259/bjrcr.20150070.
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Combined pulmonary fibrosis and emphysema as a clinicoradiologic entity: Characterization of presenting lung fibrosis and implications for survival.
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Respiratory medicine 2019; (146()):106-112 doi:10.1016/j.rmed.2018.12.003.
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Distribution of emphysema and fibrosis in idiopathic pulmonary fibrosis with coexisting emphysema.
Kinoshita Y, Watanabe K, Ishii H, et al.
Histopathology 2019; (74(7)):1103-1108 doi:10.1111/his.13831.
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Combined pulmonary fibrosis and emphysema characteristics in a Greek cohort.
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ERJ open research 2019; (5(1)) doi:10.1183/23120541.00014-2018.
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Combined pulmonary fibrosis and emphysema: How does cohabitation affect respiratory functions?
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Advances in medical sciences 2019; (64(2)):285-291 doi:10.1016/j.advms.2019.03.005.
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Disease models & mechanisms 2019; (12(5)) doi:10.1242/dmm.038711.
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Combined Pulmonary Fibrosis and Emphysema: Pulmonary Function Testing and a Pathophysiology Perspective.
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Medicina (Kaunas, Lithuania) 2019; (55(9)) doi:10.3390/medicina55090580.
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Antifibrotic therapy for fibrotic lung disease beyond idiopathic pulmonary fibrosis.
Collins BF, Raghu G
European respiratory review : an official journal of the European Respiratory Society 2019; (28(153)) doi:10.1183/16000617.0022-2019.
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Uncovering the mechanisms of exertional dyspnoea in combined pulmonary fibrosis and emphysema.
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The European respiratory journal 2020; (55(1)) doi:10.1183/13993003.01319-2019.
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Improvement of pulmonary arterial compliance by pulmonary vasodilator in pulmonary hypertension from combined pulmonary fibrosis and emphysema.
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The Gly82Ser mutation in AGER contributes to pathogenesis of pulmonary fibrosis in combined pulmonary fibrosis and emphysema (CPFE) in Japanese patients.
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Objective quantitative multidetector computed tomography assessments in patients with combined pulmonary fibrosis with emphysema: Relationship with pulmonary function and clinical events.
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Progressive fibrosing interstitial lung diseases: A new concept and indication of nintedanib.
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Combined Pulmonary Fibrosis and Emphysema (CPFE) Clinical Features and Management.
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The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation 2021; (40(6)):494-503 doi:10.1016/j.healun.2021.02.006.
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An increased risk of lung cancer in combined pulmonary fibrosis and emphysema patients with usual interstitial pneumonia compared with patients with idiopathic pulmonary fibrosis alone: a systematic review and meta-analysis.
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Clinical Features and Outcomes of Combined Pulmonary Fibrosis and Emphysema After Lung Transplantation.
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Diagnosis and Management of Idiopathic Pulmonary Fibrosis.
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Rhode Island medical journal (2013) 2021; (104(7)):26-29.
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Chronic Obstructive Pulmonary Disease Combined with Interstitial Lung Disease.
Choi JY, Song JW, Rhee CK
Tuberculosis and respiratory diseases 2022; (85(2)):122-136 doi:10.4046/trd.2021.0141.
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Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline.
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American journal of respiratory and critical care medicine 2022; (205(9)):e18-e47 doi:10.1164/rccm.202202-0399ST.
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Combined Pulmonary Fibrosis and Emphysema and Digital Clubbing.
Rasul TF, Bergholz DR, Rovinski R, et al.
Cureus 2022; (14(4)):e24231 doi:10.7759/cureus.24231.
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Syndrome of Combined Pulmonary Fibrosis and Emphysema: An Official ATS/ERS/JRS/ALAT Research Statement.
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American journal of respiratory and critical care medicine 2022; (206(4)):e7-e41 doi:10.1164/rccm.202206-1041ST.
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Outcomes of Lung Transplantation in Patients With Combined Pulmonary Fibrosis and Emphysema: A Single-Center Experience.
Narula T, Martin AK, Asif AA, et al.
Transplantation proceedings 2023; (55(2)):449-455 doi:10.1016/j.transproceed.2023.01.010.
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Connective Tissue Disease Associated Interstitial Lung Disease.
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Immunology and allergy clinics of North America 2023; (43(2)):229-244 doi:10.1016/j.iac.2023.01.005.
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An increased risk of pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema: a meta-analysis.
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BMC pulmonary medicine 2023; (23(1)):221 doi:10.1186/s12890-023-02425-4.
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Combined Pulmonary Fibrosis and Emphysema: Comparative Evidence on a Complex Condition.
Calaras D, Mathioudakis AG, Lazar Z, Corlateanu A
Biomedicines 2023; (11(6)) doi:10.3390/biomedicines11061636.
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Combined Pulmonary Fibrosis and Emphysema in a Patient With Chronic Occupational Exposure to Trichloroethylene.
Asif H, Braman SS
Military medicine 2024; (189(3-4)):e907-e910 doi:10.1093/milmed/usad359.
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Mortality surrogates in combined pulmonary fibrosis and emphysema.
Zhao A, Gudmundsson E, Mogulkoc N, et al.
The European respiratory journal 2024; (63(4)) doi:10.1183/13993003.00127-2023.
PMID: 37973176 - 46
Combined pulmonary fibrosis and emphysema.
Caceres JD, Venkata A
Current opinion in pulmonary medicine 2024; (30(2)):167-173 doi:10.1097/MCP.0000000000001044.
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Pulmonary Hypertension With Interstitial Pneumonia: Initial Treatment Effectiveness and Severity in a Japan Registry.
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JACC. Asia 2024; (4(5)):403-417 doi:10.1016/j.jacasi.2024.01.009.
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Clinical implications of six-minute walk test in patients with idiopathic pulmonary fibrosis: a retrospective cohort study.
Kim MJ, Nathan SD, Kim HH, Kim HC
Therapeutic advances in respiratory disease 2024; (18()):17534666241275329 doi:10.1177/17534666241275329.
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Incidence of and predictive factors for lung cancer in patients with rheumatoid arthritis: A retrospective long-term follow-up study.
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Modern rheumatology 2025; (35(2)):240-248 doi:10.1093/mr/roae084.
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Pirfenidone as a Cornerstone in the Management of Fibrotic Interstitial Lung Diseases and Its Emerging Applications: A Comprehensive Review.
Babariya H, Gaidhane SA, Acharya S, Kumar S
Cureus 2024; (16(9)):e70497 doi:10.7759/cureus.70497.
PMID: 39479105 - 51
The Potential Role of Cigarette Smoke, Elastic Fibers, and Secondary Lung Injury in the Transition of Pulmonary Emphysema to Combined Pulmonary Fibrosis and Emphysema.
Cantor J
International journal of molecular sciences 2024; (25(21)) doi:10.3390/ijms252111793.
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Prognostic value of a composite physiologic index developed by adding bronchial and hyperlucent volumes quantified via artificial intelligence technology.
Uyama M, Handa T, Uozumi R, et al.
Respiratory research 2024; (25(1)):442 doi:10.1186/s12931-024-03075-8.
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Emphysema or fibrosis progression in patients with combined pulmonary fibrosis and emphysema.
Zhai L, Wang F, Liu H, et al.
The American journal of the medical sciences 2025; (369(5)):562-567 doi:10.1016/j.amjms.2025.01.004.
PMID: 39788421 - 54
Association of combined pulmonary fibrosis and emphysema in rheumatoid arthritis with high titer of rheumatoid factor and autoimmunity to the lung.
Ma A, Huang R, Guo J, et al.
Frontiers in immunology 2025; (16()):1514552 doi:10.3389/fimmu.2025.1514552.
PMID: 39975554 - 55
Combined Pulmonary Fibrosis and Emphysema (CPFE): A "New" Smoking-Related Interstitial Lung Disease (ILD).
Afloarei CA, Birladeanu T, Pintilie AL, et al.
Biomedicines 2025; (13(11)) doi:10.3390/biomedicines13112703.
PMID: 41301796 - 56
Combined pulmonary fibrosis and emphysema: a narrative review.
Zeng W, Liang B, Ou X
Frontiers in medicine 2025; (12()):1683252 doi:10.3389/fmed.2025.1683252.
PMID: 41426595 - 57
Acute Exacerbation of Interstitial Lung Disease: Early Diagnosis and Treatment.
León-Román F, Martínez-Besteiro E, Iturbe D, et al.
Medicina (Kaunas, Lithuania) 2025; (61(12)) doi:10.3390/medicina61122097.
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Radiologic Pattern of Fibrosis in Combined Pulmonary Fibrosis and Emphysema: Impact on Disease Trajectories and Prognostic Outcomes.
Kim MS, Choe J, Hwang HJ, et al.
Radiology 2026; (318(2)):e251686 doi:10.1148/radiol.251686.
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Prognostic factors in combined pulmonary fibrosis and emphysema: a systematic review and meta-analysis.
Xu L, Zang D, Yan R, et al.
Journal of thoracic disease 2026; (18(2)):92 doi:10.21037/jtd-2025-aw-2054.
PMID: 41816468 - 60
Long-term follow-up results of patients with combined pulmonary fibrosis and emphysema: A single-center experience.
Calis AG, Uzer F, Gurbuz AF, et al.
Respiratory medicine 2026; (255()):108780 doi:10.1016/j.rmed.2026.108780.
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[Analysis of external respiratory function disturbances in combined pulmonary fibrosis and emphysema].
Markov NV, Khodorik NA, Ilkovich MM, et al.
Terapevticheskii arkhiv 2026; (98(3)):154-161 doi:10.26442/00403660.2026.03.203538.
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Use of Oxygen in the Emergency Department.
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Seminars in respiratory and critical care medicine 2026; doi:10.1055/a-2946-5854.
PMID: 42660530 - 63
The Efficacy of Antifibrotics in Combined Pulmonary Fibrosis and Emphysema: A Nationwide Multicentre Retrospective Cohort Study.
Gwon HR, Lee J, Chung MP, et al.
Archivos de bronconeumologia 2026; doi:10.1016/j.arbres.2026.08.001.
PMID: 42760209