Combined pulmonary fibrosis and emphysema in a smoker

There is increasing clinical, radiologic and pathologic recognition of the coexistence of emphysema and pulmonary fibrosis, resulting in a clinical syndrome known as combined pulmonary fibrosis and emphysema (CPFE). It is characterized by dyspnoea, upper-lobe emphysema, lower-lobe fibrosis and abnormalities of gas exchange. This syndrome is frequently complicated by pulmonary hypertension, acute lung injury and lung cancer. The CPFE syndrome typically occurs in male smokers, and the mortality associated with this condition, especially if pulmonary hypertension is present, is significant. Although most cases of CPFE are likely to represent the common fibrotic pattern of upper lobe fibrosis (UIP), a few cases have been reported as showing desquamative interstitial pneumonia (DIP) or unclassified interstitial pneumonia.

We present a case of CPFE which has HRCT evidence of emphysema of upper lobes and NSIP type of fibrosis in lower lobes in a smoker with normal pulmonary function.