PiSZ-phenotype alpha 1-antitrypsin deficiency: a rare cause of bronchiectasis

Panacinar emphysema is the most characteristic pulmonary disease in patients with alpha 1-antitrypsin deficiency (AAT). Bronchiectasis can also arise with AAT deficiency, although the association is much less common and no clear cause-effect relationship has yet been established. We report the case of a woman who presented with bronchiectasis and PiSZ-phenotype ATT deficiency, without emphysema. A review of the literature showed that the case is exceptional.