[Combined pulmonary fibrosis and emphysema: the natural history of the disease. The chronological evolution of clinical features, respiratory function and the CT scan]

Abstract

Introduction: The syndrome of combined basal pulmonary fibrosis and apical emphysema (CPFE) is characterised by severe dyspnoea not fully explained by pulmonary function tests that show subnormal lung volumes and expiratory flows while CT imaging reveals varying degrees of emphysema and interstitial fibrosis.

Case report: A man presented with chronic obstructive pulmonary disease associated with severe emphysema. Airflow obstruction was associated with early over-inflation. Five years later he developed interstitial fibrosis with CT appearances compatible with non-specific interstitial pneumonitis. Simultaneously, the decreased expiratory flows due to emphysema had become normal and the pulmonary distension had resolved.

Conclusion: When CPFE develops, the reduced expiratory flows and thoracic distension due to emphysema can resolve. These changes may be explained by the late development of interstitial changes, which balance the functional consequences of emphysema by an increase in elastic recoil and reduction in volume. This is the first case report that describes the evolutionary profile in a single patient. It reinforces the pathophysiological hypothesis that explains the normal pulmonary volumes and expiratory flows in CPFE.