From Wikipedia, the free encyclopedia
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