Combined Pulmonary Fibrosis and Emphysema (CPFE) Clinical Features and Management

References

• Portillo K, Morera J. Combined pulmonary fibrosis and emphysema syndrome: A new phenotype within the spectrum of smoking-related interstitial lung disease. Pulm Med. 2012. 2012. doi:10.1155/2012/867870
   PubMedGoogle Scholar
• Cottin V, Nunes H, Brillet PY, et al. Combined pulmonary fibrosis and emphysema: A distinct underrecognised entity. Eur Respir J. 2005;26(4):586–593. doi:10.1183/09031936.05.00021005
   PubMed Web of Science ®Google Scholar
• Frasca JM, Auerbach O, Parks VR, Jamieson JD. Electron microscopic observations on pulmonary fibrosis and emphysema in smoking dogs’. Experimental Molecular Pathol. 1971;16:108–125. doi:10.1016/0014-4800(71)90022-0
   Web of Science ®Google Scholar
• Auerbach O, Garfinkel L, Cuykr Hammond E. Relation of smoking and age to findings in lung parenchyma: a microscopic study. Chest. 1974;65(1):29–35. doi:10.1378/chest.65.1.29
   PubMed Web of Science ®Google Scholar
• Wiggins J, Strickland B, Turner-Warwick M. Combined cryptogenic fibrosing alveolitis and emphysema: the value of high resolution computed tomography in assessment. Respir Med. 1990;84(5):365–369. doi:10.1016/S0954-6111(08)80070-4
   PubMed Web of Science ®Google Scholar
• Mejía M, Carrillo G, Rojas-Serrano J, et al. Idiopathic pulmonary fibrosis and emphysema: decreased survival associated with severe pulmonary arterial hypertension. Chest. 2009;136(1):10–15. doi:10.1378/chest.08-2306
   PubMed Web of Science ®Google Scholar
• Kurashima K, Takayanagi N, Tsuchiya N, et al. The effect of emphysema on lung function and survival in patients with idiopathic pulmonary fibrosis. Respirology. 2010;15(5):843–848. doi:10.1111/j.1440-1843.2010.01778.x
   PubMed Web of Science ®Google Scholar
• Jankowich MD, Rounds S. Combined pulmonary fibrosis and emphysema alters physiology but has similar mortality to pulmonary fibrosis without emphysema. Lung. 2010;188(5):365–373. doi:10.1007/s00408-010-9251-6
   PubMed Web of Science ®Google Scholar
• Hiwatari N, Shimura S, Takishima T. Pulmonary emphysema followed by pulmonary fibrosis of undetermined cause. Respiration. 1993;60(6):354–358. doi:10.1159/000196235
   PubMed Web of Science ®Google Scholar
• Bédard Méthot D, Leblanc É, Lacasse Y. Meta-analysis of gastroesophageal reflux disease and idiopathic pulmonary fibrosis. Chest. 2019;155(1):33–43. doi:10.1016/j.chest.2018.07.038
   PubMed Web of Science ®Google Scholar
• Tzouvelekis A, Zacharis G, Oikonomou A, et al. Increased incidence of autoimmune markers in patients with combined pulmonary fibrosis and emphysema. BMC Pulm Med. 2013;13:1. doi:10.1186/1471-2466-13-31
   PubMed Web of Science ®Google Scholar
• Cottin V, Nunes H, Mouthon L, et al. Combined pulmonary fibrosis and emphysema syndrome in connective tissue disease. Arthritis Rheum. 2011;63(1):295–304. doi:10.1002/art.30077
   PubMed Web of Science ®Google Scholar
• Collum SD, Molina JG, Hanmandlu A, et al. Adenosine and hyaluronan promote lung fibrosis and pulmonary hypertension in combined pulmonary fibrosis and emphysema. DMM. 2019;12:5. doi:10.1242/dmm.038711
   Web of Science ®Google Scholar
• Epaud R, Delestrain C, Louha M, Simon S, Fanen P, Tazi A. Combined pulmonary fibrosis and emphysema syndrome associated with ABCA3 mutations. Eur Respir J. 2014;43(2):638–641. doi:10.1183/09031936.00145213
   PubMed Web of Science ®Google Scholar
• Lundblad LKA, Thompson-Figueroa J, Leclair T, et al. Tumor necrosis factor-α overexpression in lung disease: A single cause behind a complex phenotype. Am J Respir Crit Care Med. 2005;171(12):1363–1370. doi:10.1164/rccm.200410-1349OC
   PubMed Web of Science ®Google Scholar
• Fulkerson PC, Fischetti CA, Hassman LM, Nikolaidis NM, Rothenberg ME. Persistent effects induced by IL-13 in the lung. Am J Respir Cell Mol Biol. 2006;35(3):337–346. doi:10.1165/rcmb.2005-0474OC
   PubMed Web of Science ®Google Scholar
• Sakai F, Tominaga J, Kaga A, et al. Imaging diagnosis of interstitial pneumonia with emphysema (combined pulmonary fibrosis and emphysema). Pulm Med. 2012;2012:1–9. doi:10.1155/2012/816541;.
   Google Scholar
• Alsumrain M, de Giacomi F, Nasim F, et al. Combined pulmonary fibrosis and emphysema as a clinicoradiologic entity: characterization of presenting lung fibrosis and implications for survival. Respir Med. 2019;146:106–112. doi:10.1016/j.rmed.2018.12.003
   PubMed Web of Science ®Google Scholar
• Devaraj A, Wells AU, Meister MG, Corte TJ, Hansell DM. The effect of diffuse pulmonary fibrosis on the reliability of CT signs of pulmonary hypertension. Radiology. 2008;249(3):1042–1049. doi:10.1148/radiol.2492080269
   PubMed Web of Science ®Google Scholar
• Inomata M, Ikushima S, Awano N, et al. An autopsy study of combined pulmonary fibrosis and emphysema: correlations among clinical, radiological, and pathological features. BMC Pulm Med. 2014;14:1. doi:10.1186/1471-2466-14-104
   PubMed Web of Science ®Google Scholar
• Wells AU, Desai SR, Rubens MB, et al. Idiopathic pulmonary fibrosis: A composite physiologic index derived from disease extent observed by computed tomography. Am J Respir Crit Care Med. 2003;167(7):962–969. doi:10.1164/rccm.2111053
   PubMed Web of Science ®Google Scholar
• Schmidt SL, Nambiar AM, Tayob N, et al. Pulmonary function measures predict mortality differently in IPF versus combined pulmonary fibrosis and emphysema. Eur Respir J. 2011;38(1):176–183. doi:10.1183/09031936.00114010
   PubMed Web of Science ®Google Scholar
• Heathcote KL, Cockcroft DW, Fladeland DA, Fenton ME. Normal expiratory flow rate and lung volumes in patients with combined emphysema and interstitial lung disease: a case series and literature review case presentation. Can Respir J. 2011;18(5):e73–76. doi:10.1155/2011/354325.
   Google Scholar
• Sato T, Tsujino I, Tanino M, Ohira H, Nishimura M. Broad and heterogeneous vasculopathy in pulmonary fibrosis and emphysema with pulmonary hypertension. Respirol Case Rep. 2013;1(1):10–13. doi:10.1002/rcr2.7
   PubMedGoogle Scholar
• Cottin V, le Pavec J, Prévot G, et al. Pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema syndrome. Eur Respir J. 2010;35(1):105–111. doi:10.1183/09031936.00038709
   PubMed Web of Science ®Google Scholar
• Dotan Y, Stewart J, Gangemi A, et al. Pulmonary vasculopathy in explanted lungs from patients with interstitial lung disease undergoing lung transplantation. BMJ Open Respir Res. 2020;7:1. doi:10.1136/bmjresp-2019-000532
   Web of Science ®Google Scholar
• Zantah M, Dotan Y, Dass C, Zhao H, Marchetti N, Criner GJ. Acute exacerbations of COPD versus IPF in patients with combined pulmonary fibrosis and emphysema. Respir Res. 2020;21:1. doi:10.1186/s12931-020-01432-x
   PubMed Web of Science ®Google Scholar
• Collard HR, Ryerson CJ, Corte TJ, et al. Acute exacerbation of idiopathic pulmonary fibrosis an international working group report. Am J Respir Crit Care Med. 2016;194(3):265–275. doi:10.1164/rccm.201604-0801CI
   PubMed Web of Science ®Google Scholar
• Ikuyama Y, Ushiki A, Kosaka M, et al. Prognosis of patients with acute exacerbation of combined pulmonary fibrosis and emphysema: A retrospective single-centre study. BMC Pulm Med. 2020;20:1. doi:10.1186/s12890-020-01185-9
   PubMed Web of Science ®Google Scholar
• Kitaguchi Y, Fujimoto K, Hanaoka M, Kawakami S, Honda T, Kubo K. Clinical characteristics of combined pulmonary fibrosis and emphysema. Respirology. 2010;15(2):265–271. doi:10.1111/j.1440-1843.2009.01676.x
   PubMed Web of Science ®Google Scholar
• Koo HJ, Do KH, Lee JB, Alblushi S, Lee SM. Lung cancer in combined pulmonary fibrosis and emphysema: A systematic review and meta-analysis. PLoS One. 2016;11:9. doi:10.1371/journal.pone.0161437
   Web of Science ®Google Scholar
• Li C, Wu W, Chen N, et al. Clinical characteristics and outcomes of lung cancer patients with combined pulmonary fibrosis and emphysema: A systematic review and meta-analysis of 13 studies. J Thorac Dis. 2017;9(12):5322–5334. doi:10.21037/jtd.2017.12.72
   PubMed Web of Science ®Google Scholar
• Raghu G, Remy-Jardin M, Myers JL, et al. Diagnosis of idiopathic pulmonary fibrosis. an official ATS/ERS/JRS/ALAT clinical practice guideline. American J Respir Critical Care Med. 2018;198:e44–e68. doi:10.1164/rccm.201807-1255ST
   PubMed Web of Science ®Google Scholar
• Wells AU. “Any fool can make a rule and any fool will mind it.”. BMC Med. 2016;14:1. doi:10.1186/s12916-016-0562-1
   PubMed Web of Science ®Google Scholar
• Lentz RJ, Taylor TM, Kropski JA, et al. Utility of flexible bronchoscopic cryobiopsy for diagnosis of diffuse parenchymal lung diseases. J Bronchology Interv Pulmonol. 2018;25(2):88–96. doi:10.1097/LBR.0000000000000401
   PubMed Web of Science ®Google Scholar
• Dong F, Zhang Y, Chi F, et al. Clinical efficacy and safety of ICS/LABA in patients with combined idiopathic pulmonary fibrosis and emphysema. Int J Clin Exp Med. 2015;8(6):8617–8625.
   PubMed Web of Science ®Google Scholar
• Sogkas G, Hirsch S, Olsson KM, et al. Lung involvement in primary sjögren’s syndrome—an under-diagnosed entity. Front Med. 2020;7:7. doi:10.3389/fmed.2020.00332
   Web of Science ®Google Scholar
• Straub G, Caviezel C, Frauenfelder T, Bloch KE, Franzen D. Successful lung volume reduction surgery in combined pulmonary emphysema and fibrosis without body-plethysmographic hyperinflation-a case report. J Thorac Dis. 2018;10:S2830–S2834. doi:10.21037/jtd.2018.06.60
   PubMed Web of Science ®Google Scholar
• King TE, Bradford WZ, Castro-Bernardini S, et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. New England J Med. 2014;370(22):2083–2092. doi:10.1056/nejmoa1402582
   PubMed Web of Science ®Google Scholar
• Cottin V, Azuma A, Raghu G, et al. Therapeutic effects of nintedanib are not influenced by emphysema in the Inpulsis trials. Eur Respir J. 2019;53:4. doi:10.1183/13993003.01655-2018
   Web of Science ®Google Scholar
• Richeldi L, Du Bois RM, Raghu G, et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. New England J Med. 2014;370(22):2071–2082. doi:10.1056/nejmoa1402584
   PubMed Web of Science ®Google Scholar
• Flaherty KR, Wells AU, Cottin V, et al. Nintedanib in progressive fibrosing interstitial lung diseases. New England J Med. 2019;381(18):1718–1727. doi:10.1056/NEJMoa1908681
   PubMed Web of Science ®Google Scholar
• Cottin V, Cordier JF, Wells AU. Centrilobular emphysema combined with pulmonary fibrosis results in improved survival: A response. Fibrogenesis Tissue Repair. 2011;4:1. doi:10.1186/1755-1536-4-16
   PubMedGoogle Scholar