Objective: To describe trends of reported α1-antitrypsin deficiency mortality in the United States from 1979-1991.

Methods: We analyzed death certificate reports in the multiple-cause mortality files compiled by the National Center for Health Statistics.

Results: Of the 26,866,600 deaths that occurred during the 13-year period, 1,930 had α1-antitrypsin deficiency listed as a cause of death. Over this period, we would have expected 5,400 to 13,400 persons with this condition to die. The age-adjusted mortality rate with reported α1-antitrypsin deficiency listed increased 86%, from 4.3 per 10 million in 1979 to 8.0 per 10 million in 1991. α1-Antitrypsin deficiency mortality rates were higher among whites than among blacks or persons of other races. α1-Antitrypsin deficiency was listed in 2.7% of all deaths with obstructive lung disease among persons aged 35-44 years old and in 1.2% of all deaths listing hepatic disease among children aged 1 to 14 years old.

Conclusions: α1-Antitrypsin deficiency is an important risk factor for obstructive lung disease and hepatic disease in the United States, and it was reported with increasing frequency through the study period, although it is still likely underreported.