Combined pulmonary fibrosis with emphysema (CPFE) is, literally, a combination of these two entities but with confusing clinical findings, because lung volumes are preserved while diffusing capacity is markedly decreased. At a microscopic level, CPFE typically shows fairly large emphysematous spaces and a fibrosing interstitial pneumonia that is hard to diagnose because the emphysematous spaces distort the architecture. CPFE is most commonly seen with UIP but sometimes also with other forms of fibrosing interstitial pneumonias. CPFE patients have a high incidence of lung cancer and pulmonary hypertension.