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

Figures & data

Table 1 Studies on Patients with Combined Pulmonary Fibrosis and Emphysema (CPFE)

Author, Year	Number of Patients with CPFE	Mean Age	Male/Female	Smoking (PY)	Specific Aspects	5-Year Survival
Wiggins, 1990^Citation5	8	NR	NR	NR	First description of CPFE type disease; current or former heavy smokers	NR
Hiwatari, 1993^Citation9	9	NR	9 (100%) Male	NR	Current or former heavy smokers	NR
Cottin, 2005^Citation2	61	65.2±10.2	60 (98%) Male	46 ± 27	First definition of the term “CPFE”	54.6%*
Zhang, 2016^Citation38	87	66±8.5	76 (87%) Male	NR	50% Wheezing; less viral infections than IPF	43.2%
Jankowich, 2010^Citation8	20	69±10	20 (100%) Male	NR	100% Former smokers	35%
Mejía, 2009^Citation6	31	67±7	30 (97%) Male	5 (0–60)		NR
Akagi, 2009^Citation39	26	65±18.5	23 (89%) Male	37.5
Kurashima, 2010^Citation7	221	72.9±8.1	209 (95%) Male	55.3 ± 24.9		NR
Ryerson, 2013^Citation40	29	69.9±8.7	20 (69%) Male	46.4 ± 15.3		NR
Sugino, 2014^Citation41	46	71.4±6.7	43 (94%) Male			NR
Kim, 2014^Citation42	26	67.6±2.2	23 (89%) Male	28.3 ± 4.1		NR
Sato, 2016^Citation43	55	71.8±7.3	53 (96%) Male	NR		NR
Papaioannou, 2016^49	29	75±13	26 (90%) Male	NR		NR
Kohashi, 2016^50	34	62±26	Unknown	NR		NR
Portillo, 2017^51	29	NR	29 (100%) Male	NR		NR
Alsumrain, 2019^Citation18	179	69.6±9.3	139 (78%)	50 ± 30		NR

Note: *87.5% 2-year survival.

Abbreviations: CPFE, combined pulmonary fibrosis and emphysema; NR, not reported; PY, pack-years.

Figure 1 Clinicoradiological interstitial lung diseases in CPFE.

Abbreviations: cHP, chronic hypersensitivity pneumonitis; CPFE, combined pulmonary fibrosis and emphysema; CTD-ILD, connective tissue disease interstitial lung disease; DIP, desquamative interstitial pneumonia; MCTD, mixed connective tissue disease; NSIP, non-specific interstitial pneumonia; RA-ILD, rheumatoid arthritis–associated interstitial lung disease; RB-ILD, respiratory bronchiolitis interstitial lung disease; Ssc, systemic sclerosis.

Figure 2 Diagnosis and management of patients with interstitial lung diseases in CPFE.

Abbreviations: BAL, bronchoalveolar lavage; BLVR, bronchoscopic lung volume reduction; CT, computed tomography; DLCO, diffusing capacity of the lung; FEV1, forced expiratory volume in 1 second; FVC, forced vital capacity; ICS, inhaled corticosteroids; LVRS, lung volume reduction surgery; NT-pro-BNP, N-terminal prohormone brain natriuretic peptide; RV, residual volume; TLC, total lung capacity.

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

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

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

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

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

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

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

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

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

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