Combined pulmonary fibrosis and emphysema explained

Combined pulmonary fibrosis and emphysema
Synonyms:	CPFE
Field:	pulmonology

Combined pulmonary fibrosis and emphysema (CPFE), describes a medical syndrome involving both pulmonary fibrosis and emphysema.^{[1] [2]} The combination is most commonly found in male smokers. Pulmonary function tests typically show preserved lung volume with very low transfer factor.^[3] __TOC__

Presentation

CFPE is characterised by shortness of breath, and reduced oxygen concentration (reflecting gas exchange abnormalities). Imaging shows upper-lobe emphysema, and lower-lobe interstitial fibrosis.^[4] CFPE is often complicated by pulmonary hypertension, acute lung injury, lung cancer, and coronary artery disease.^[4]

Diagnosis

The diagnosis is confirmed with high resolution CT scan.^[5]

Notes and References

1. Cottin . V . Cordier JF . Combined pulmonary fibrosis and emphysema: an experimental and clinically relevant phenotype . American Journal of Respiratory and Critical Care Medicine . 172 . 12 . 1605; author reply 1605–6 . June 2005 . 16339012 . 10.1164/ajrccm.172.12.1605a . 2009-02-23 . 2011-09-27 . https://web.archive.org/web/20110927232444/http://ajrccm.atsjournals.org/cgi/content/full/172/12/1605a . dead .
2. Cottin . V . Nunes H . Brillet PY . Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity . European Respiratory Journal . 26 . 4 . 586–593 . February 2006 . 16204587 . 10.1183/09031936.05.00021005 . etal. free .
3. Papiris . SA . Triantafillidou . C . Manali . ED . Combined pulmonary fibrosis and emphysema . Expert Review of Respiratory Medicine . 7 . 1 . 19–31 . February 2013 . 23362797 . 10.1586/ers.12.80 . 37007269 .
4. Wand. O. Kramer. MR. January 2018. Harefuah. 157. 1. 28–33. 29374870. The Syndrome of Combined Pulmonary Fibrosis and Emphysema - CPFE.
5. Jankowich . MD . Rounds . SI . Combined pulmonary fibrosis and emphysema syndrome: a review . Chest . 141 . 1 . 222–231 . January 2012 . 22215830 . 3251269 . 10.1378/chest.11-1062 .