The Lungs in Homozygous Alpha1Antitrypsin Deficiency

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Abstract
A morphologic study of three lungs from patients with homozygous deficiency of serum alpha1 antitrypsin was undertaken to determine the presence, type, and distribution of emphysema and the coexistence, if any, of airway disease in the form of chronic bronchitis or bronchiolitis. All three lungs had panlobular emphysema, involving most severely the dependent portions of the lower lobes and to a lesser extent the upper lobes. The lungs also showed microscopic features of chronic bronchitis. The three lungs were from patients who had been cigarette smokers. Previous reports concerning the association of serum alpha1 antitrypsin deficiency and chronic obstructive pulmonary disease have emphasized the panlobular emphysema and may have overlooked accompanying coexistent chronic bronchitis. The exact effect of cigarette smoking in antitrypsin deficiency remains to be defined, but it apparently accelerates the development of emphysema and is also involved in the pathogenesis of coexistent chronic bronchitis. The bronchioles studied showed remarkably little airway disease.

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