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Combined pulmonary fibrosis and emphysema


FieldValue
nameCombined pulmonary fibrosis and emphysema
synonymsCPFE
fieldpulmonology

Combined pulmonary fibrosis and emphysema (CPFE), describes a medical syndrome involving both pulmonary fibrosis and emphysema. The combination is most commonly found in male smokers. Pulmonary function tests typically show preserved lung volume with very low transfer factor. 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. CFPE is often complicated by pulmonary hypertension, acute lung injury, lung cancer, and coronary artery disease.

Diagnosis

The diagnosis is confirmed with high resolution CT scan.

References

References

  1. Cottin, V. (June 2005). "Combined pulmonary fibrosis and emphysema: an experimental and clinically relevant phenotype". American Journal of Respiratory and Critical Care Medicine.
  2. Cottin, V. (February 2006). "Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity". European Respiratory Journal.
  3. (February 2013). "Combined pulmonary fibrosis and emphysema". Expert Review of Respiratory Medicine.
  4. (January 2018). "The Syndrome of Combined Pulmonary Fibrosis and Emphysema - CPFE". Harefuah.
  5. (January 2012). "Combined pulmonary fibrosis and emphysema syndrome: a review". Chest.
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