Alpha-1-antitrypsin deficiency presenting as bronchiectasis.

We describe three patients with the clinical and radiographic features of progressive bronchiectasis, and without evidence of emphysema, associated with deficiency of alpha-1-antitrypsin. The association of hereditary alpha-1-antitrypsin deficiency and pulmonary emphysema is well recognized (Eriksson 1965). About half of these subjects also have chronic bronchitis as defined by persistent sputum production (Tobin et al. 1983). Bronchographically proven bronchiectasis without apparent emphysema has been reported in only one subject with alpha-1-antitrypsin deficiency (Longstretch et al. 1975).