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.
The presence of characteristic imaging features in the correct clinical context is very suggestive of the diagnosis. HRCT would typically show:
centrilobular and/or paraseptal emphysema:
- often upper zone predominant 5
- the emphysema competent may have tendency to being more paraseptal 9
- pulmonary fibrosis of the lower lobes: can be of UIP or NSIP pattern
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
Treatment and prognosis
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.
Patients may have a higher risk of developing lung cancer 11. Lung cancer occurring on those with CPFE may have a poorer prognosis and according to one study, the commonest type has been squamous cell lung cancer 10.
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- 6. Brillet PY, Cottin V, Letoumelin P et-al. [Combined apical emphysema and basal fibrosis syndrome (emphysema/fibrosis syndrome): CT imaging features and pulmonary function tests]. J Radiol. 2009;90 (1 Pt 1): 43-51. J Radiol (link) - Pubmed citation
- 7. Kitaguchi Y, Fujimoto K, Hanaoka M et-al. Pulmonary function impairment in patients with combined pulmonary fibrosis and emphysema with and without airflow obstruction. Int J Chron Obstruct Pulmon Dis.9: 805-11. doi:10.2147/COPD.S65621 - Free text at pubmed - Pubmed citation
- 8. Kitaguchi Y, Fujimoto K, Hayashi R et-al. Annual changes in pulmonary function in combined pulmonary fibrosis and emphysema: over a 5-year follow-up. Respir Med. 2013;107 (12): 1986-92. doi:10.1016/j.rmed.2013.06.015 - Pubmed citation
- 9. Ciccarese F, Attinà D, Zompatori M. Combined pulmonary fibrosis and emphysema (CPFE): what radiologist should know. (2016) La Radiologia medica. 121 (7): 564-72. doi:10.1007/s11547-016-0627-4 - Pubmed
- 10. Koo HJ, Do KH, Lee JB, Alblushi S, Lee SM. Lung Cancer in Combined Pulmonary Fibrosis and Emphysema: A Systematic Review and Meta-Analysis. (2016) PloS one. 11 (9): e0161437. doi:10.1371/journal.pone.0161437 - Pubmed
- 11. Papaioannou AI, Kostikas K, Manali ED, Papadaki G, Roussou A, Kolilekas L, Borie R, Bouros D, Papiris SA. Combined pulmonary fibrosis and emphysema: The many aspects of a cohabitation contract. (2016) Respiratory medicine. 117: 14-26. doi:10.1016/j.rmed.2016.05.005 - Pubmed