Combined pulmonary fibrosis and emphysema

Combined pulmonary fibrosis and emphysema (CPFE) are a possible new addition to a growing list of smoking-related lung disease characterised by the coexistence of usual interstitial pneumonia (UIP) or nonspecific interstitial pneumonia (NSIP) with emphysema in smokers.

It typically occurs in male smokers 3. It is estimated to represent between 5-10% of cases of diffuse interstitial lung disease.

 Clinical features include:

  • tobacco smoking
  • severe dyspnoea
  • unexpected subnormal spirometry findings
  • severely impaired transfer capacity for carbon monoxide
  • hypoxaemia on exercise

Although the pathogenesis and the exact association are yet to be elucidated in larger studies, it is important to be aware of probable inherent susceptibility of some patient to pulmonary fibrosis from cigarette smoking.  

CT

The presence of characteristic imaging features in the correct clinical context is very suggestive of the diagnosis. HRCT would typically show:

Three distinct patterns have been described in recent literature 6:

  • progressive transition with diffuse emphysema (centrilobular and/or bullous) and zone of transition between bullae and honeycombing
  • paraseptal emphysema with predominant subpleural bullae of enlarging size at the bases
  • separate processes with independent areas of fibrosis and emphysema
Complications

The risk of the development of pulmonary hypertension is considered to be notably higher in those patients with CPFE than in those with idiopathic pulmonary fibrosis without emphysema 1-2.

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Article information

rID: 19021
System: Chest
Section: Pathology
Synonyms or Alternate Spellings:
  • Combined pulmonary fibrosis and emphysema (CPFE)
  • Combined pulmonary fibrosis with emphysema
  • CPFE
  • CPFE syndrome
  • Syndrome of combined pulmonary fibrosis and emphysema
  • Syndrome of combined pulmonary fibrosis with emphysema

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Cases and figures

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    Case 1: combined NSIP and emphysema in smoker
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    Case 2: combined UIP with emphysema in smoker
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    Case 3: probable
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