Combined Pulmonary Fibrosis and Emphysema: A Case Report and Literature Review

Document Type : Case Report

Authors

1 Internal Medicine, Department of Internal Medicine, Faculty of Medicine, Mashhad University of Medical Science, Mashhad, Iran

2 Pulmonologist, Lung Diseases Research Center, Mashhad University of Medical Science, Mashhad, Iran.

Abstract

The syndrome of combined emphysema of the upper lobes and fibrosis of the lower lobes on chest computed tomography results in a characteristic functional profile, with preserved lung volumes, hypoxemia at exercise. Despite subnormal spirometry, combined pulmonary fibrosis and emphysema is a severe entity. The presence of pulmonary arterial hypertension at diagnosis is a critical determinant of prognosis.

Keywords


1.             Lin H, Jiang S. Combined pulmonary fibrosis and emphysema (CPFE): an entity different from emphysema or pulmonary fibrosis alone. Journal of thoracic disease. 2015;7(4):767.
2.             Hasani, S., Mozdourian, M. Chronic obstructive pulmonary disease and bone health: A review on possible links and follow up studies. Journal of Cardio-Thoracic Medicine, 2020; 8(4): 684-692
3.             Jankowich MD, Polsky M, Klein M, Rounds S. Heterogeneity in combined pulmonary fibrosis and emphysema. Respiration. 2008;75(4):411-7.
4.             Cottin V, Brillet P-Y, Nunes H, Cordier J-F. Combined pulmonary fibrosis and emphysema. Presse medicale (Paris, France: 1983). 2007;36(6 Pt 2):936-44.
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6.             Katzenstein A-LA, Myers JL. Idiopathic pulmonary fibrosis: clinical relevance of pathologic classification. American journal of respiratory and critical care medicine. 1998;157(4):1301-15.
7.             Jankowich MD, Rounds SIS. Combined pulmonary fibrosis and emphysema syndrome: a review. Chest. 2012;141(1):222-31.
8.             Marten K, Milne D, Antoniou KM, Nicholson AG, Tennant RC, Hansel TT, et al. Non-specific interstitial pneumonia in cigarette smokers: a CT study. European radiology. 2009;19(7):1679-85.
9.             Cottin V, Cordier J-F. The syndrome of combined pulmonary fibrosis and emphysema. Chest. 2009;136(1):1-2.
10.          Kitaguchi Y, Fujimoto K, Hanaoka M, Kawakami S, Honda T, Kubo K. Clinical characteristics of combined pulmonary fibrosis and emphysema. Respirology. 2010;15(2):265-71.
11.          Odani K, Yoriko M, Shoji Y. Computed tomographic evaluation in the cases of coexistence of pulmonary emphysema with idiopathic pulmonary fibrosis. Journal of Tomography (Japan). 2004;31(1):25-9.
12.          Hiwatari N, Shimura S, Takishima T. Pulmonary emphysema followed by pulmonary fibrosis of undetermined cause. Respiration. 1993;60(6):354-8.
13.          Ryerson CJ, Hartman T, Elicker BM, Ley B, Lee JS, Abbritti M, et al. Clinical features and outcomes in combined pulmonary fibrosis and emphysema in idiopathic pulmonary fibrosis. Chest. 2013;144(1):234-40.
14.          Cottin V, Nunes H, Brillet P, Delaval P, Devouassoux G, Tillie-Leblond I, et al. Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. European Respiratory Journal. 2005;26(4):586-93.
15. Chamani J. Energetic domains analysis of bovine α-lactalbumin upon interaction with copper and dodecyl trimethylammonium bromide. Journal of Molecular Structure. 2010;979(1-3)227-234.
16.          Neghab M, Rahimi E, Emad A, Fard ARR. An epidemiological study of talc-related respiratory morbidity among employees of a rubber industry in Shiraz-Iran. International archives of occupational and environmental health. 2007;80(6):539-46.
 
 
1.             Lin H, Jiang S. Combined pulmonary fibrosis and emphysema (CPFE): an entity different from emphysema or pulmonary fibrosis alone. Journal of thoracic disease. 2015;7(4):767.
2.             Hasani, S., Mozdourian, M. Chronic obstructive pulmonary disease and bone health: A review on possible links and follow up studies. Journal of Cardio-Thoracic Medicine, 2020; 8(4): 684-692
3.             Jankowich MD, Polsky M, Klein M, Rounds S. Heterogeneity in combined pulmonary fibrosis and emphysema. Respiration. 2008;75(4):411-7.
4.             Cottin V, Brillet P-Y, Nunes H, Cordier J-F. Combined pulmonary fibrosis and emphysema. Presse medicale (Paris, France: 1983). 2007;36(6 Pt 2):936-44.
5.             American Thoracic S. Chronic bronchitis, asthma, and pulmonary emphysema: a statement by the Committee on Diagnostic Standards for Nontuberculous Respiratory Diseases. Am Rev Respir Dis. 1962;85:762-28.
6.             Katzenstein A-LA, Myers JL. Idiopathic pulmonary fibrosis: clinical relevance of pathologic classification. American journal of respiratory and critical care medicine. 1998;157(4):1301-15.
7.             Jankowich MD, Rounds SIS. Combined pulmonary fibrosis and emphysema syndrome: a review. Chest. 2012;141(1):222-31.
8.             Marten K, Milne D, Antoniou KM, Nicholson AG, Tennant RC, Hansel TT, et al. Non-specific interstitial pneumonia in cigarette smokers: a CT study. European radiology. 2009;19(7):1679-85.
9.             Cottin V, Cordier J-F. The syndrome of combined pulmonary fibrosis and emphysema. Chest. 2009;136(1):1-2.
10.          Kitaguchi Y, Fujimoto K, Hanaoka M, Kawakami S, Honda T, Kubo K. Clinical characteristics of combined pulmonary fibrosis and emphysema. Respirology. 2010;15(2):265-71.
11.          Odani K, Yoriko M, Shoji Y. Computed tomographic evaluation in the cases of coexistence of pulmonary emphysema with idiopathic pulmonary fibrosis. Journal of Tomography (Japan). 2004;31(1):25-9.
12.          Hiwatari N, Shimura S, Takishima T. Pulmonary emphysema followed by pulmonary fibrosis of undetermined cause. Respiration. 1993;60(6):354-8.
13.          Ryerson CJ, Hartman T, Elicker BM, Ley B, Lee JS, Abbritti M, et al. Clinical features and outcomes in combined pulmonary fibrosis and emphysema in idiopathic pulmonary fibrosis. Chest. 2013;144(1):234-40.
14.          Cottin V, Nunes H, Brillet P, Delaval P, Devouassoux G, Tillie-Leblond I, et al. Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. European Respiratory Journal. 2005;26(4):586-93.
15.          Neghab M, Rahimi E, Emad A, Fard ARR. An epidemiological study of talc-related respiratory morbidity among employees of a rubber industry in Shiraz-Iran. International archives of occupational and environmental health. 2007;80(6):539-46.