Combined pulmonary fibrosis and emphysema

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 namesCPFE

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]

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


  1. 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.
  2. 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.
  3. 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.
  4. Wand, O; Kramer, MR (January 2018). "The Syndrome of Combined Pulmonary Fibrosis and Emphysema - CPFE". Harefuah. 157 (1): 28–33. PMID 29374870.
  5. 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.
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