Severe, early-onset emphysema with normal alphal-antitrypsin levels in nonsmokers: a clinical dilemma.

Approximately 85% of all cases of chronic obstructive pulmonary disease are attributed to cigarette smoking. The only other established risk factor for the development of chronic obstructive pulmonary disease that is of comparable importance is the deficiency of alpha1-antitrypsin, a rare genetic defect that is present in less than 1% of all cases. Other risk factors are not well characterized in the literature. This article describes one patient without a significant smoking history and with a normal alphaa-antitrypsin level who developed severe early-onset emphysema and gives a brief discussion about other genetic and environmental risk factors for the development of emphysema.