Which of the following statements is true?

FVC is often more compromised in CPFE than in IPF alone

There is a gap between preserved lung function and significantly comprised DLCO

CPFE is a syndrome usually found in non-smokers

Pulmonary hypertension is a rare finding in CPFE

Lung function values, especially FVC, are preserved in CPFE. This is thought to be the consequence of two counteracting disease patterns: pulmonary fibrosis restricts the lung volumes while emphysema hyperinflates them. The gas exchange, however, is significantly decreased in CPFE patients compared to IPF patients due to the combination of fibrotic and emphysematous lung disease and in some cases also due to PH.

Jankowich MD, Rounds SI. Combined pulmonary fibrosis and emphysema syndrome: a review. Chest. 2012;141(1):222-231. 
Provided by Prof. Heussel and PD Kreuter (Thoraxklinik Heidelberg, Germany)
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