Medical condition
Combined pulmonary fibrosis and emphysema Other names CPFE Specialty 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]
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]
References
^ Cottin, V; Cordier JF (June 2005). "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. doi :10.1164/ajrccm.172.12.1605a . PMID 16339012 . Archived from the original on 2011-09-27. Retrieved 2009-02-23 .
^ Cottin, V; Nunes H; Brillet PY; et al. (February 2006). "Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity" . European Respiratory Journal . 26 (4): 586–593. doi :10.1183/09031936.05.00021005 . PMID 16204587 .
^ Papiris, SA; Triantafillidou, C; Manali, ED (February 2013). "Combined pulmonary fibrosis and emphysema". Expert Review of Respiratory Medicine . 7 (1): 19–31. doi :10.1586/ers.12.80 . PMID 23362797 . S2CID 37007269 .
^ a b Wand, O; Kramer, MR (January 2018). "The Syndrome of Combined Pulmonary Fibrosis and Emphysema - CPFE". Harefuah . 157 (1): 28–33. PMID 29374870 .
^ Jankowich, MD; Rounds, SI (January 2012). "Combined pulmonary fibrosis and emphysema syndrome: a review" . Chest . 141 (1): 222–231. doi :10.1378/chest.11-1062 . PMC 3251269 . PMID 22215830 .
External links
Classification External resources