A 30-year perspective on alpha 1-antitrypsin deficiency

The early history of alpha 1-antitrypsin deficiency, in the years between 1962 and 1965, and its impact on the understanding of emphysema abnormalities were reviewed by me in 1989. This report discusses the disease spectrum (including emphysema, liver, and vasculitic diseases) and focuses on the variable clinical expression of the deficiency state. Some new findings that may be relevant to the function of alpha 1-antitrypsin are also considered.