Bronchiectasis in patients with alpha 1-antitrypsin deficiency. A rare occurrence?

Abstract

The chest radiographs and computed tomographic (CT) scans of seven patients with homozygous proteinase inhibitor phenotype Z (PiZZ) alpha 1-antitrypsin deficiency were reviewed. All patients except one showed severe emphysema with or without bullous change. Bronchiectasis was detected in three patients by CT but only in two patients by chest radiography. A young patient developed bronchiectasis before symptomatic emphysema. We stress that patients with PiZZ are susceptible to bronchiectasis, and the widespread use of CT should reveal its true incidence which might not be as low as generally believed.