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]
| Combined pulmonary fibrosis and emphysema | |
|---|---|
| Other names | CPFE |
| Specialty | Pulmonology |
Presentation
editCFPE 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
editThe diagnosis is confirmed with high resolution CT scan.[5]
References
edit- ^ 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.