CPFE is characterised by:

Only basal honeycombing

Centrilobular and/or paraseptal emphysema in upper zone and predominant pulmonary fibrosis of the lower lobes (UIP or NSIP pattern possible)

NSIP pattern

Centrilobular nodules with emphysema at upper lobes

Correct answer:
Centrilobular and/or paraseptal emphysema in upper zone and predominant pulmonary fibrosis of the lower lobes (UIP or NSIP pattern possible)


The HRCT diagnosis of CPFE is possible only if there is an association of UIP or NSIP with emphysema
The major difficulty is to distinguish between small bubbles from UIP cysts, especially when the distribution is not uniform and typical.
The honeycombing appearance of the cysts is helpful.
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