What are the histologic findings in alpha1-antitrypsin deficiency (AATD)?

Updated: Sep 11, 2020
  • Author: Dora E Izaguirre Anariba, MD, MPH; Chief Editor: John J Oppenheimer, MD  more...
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All forms of emphysema destroy alveolar walls and leave permanent abnormal enlargement of the airspace distal to the terminal bronchiole. In alpha1-antitrypsin deficiency (AATD), the emphysematous areas are distributed uniformly throughout the acinus (lobule) and, for reasons that are not known, more commonly in the basilar portions of the lung. This contrasts with centrilobular emphysema characteristic of cigarette smoking, which predominantly affects the respiratory bronchioles in the central portion of the lobule, initially at the apex of the lung.

Pathologic features from liver biopsies have shown to be nonspecific and variable depending on the classification and staging of the disease. The presence of PAS-positive globules may suggest AATD, and further investigation it is recommended.

Biopsy findings cannot replace IEF as a mode of diagnosis.

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