Relationship of leukocyte elastase concentration to severity of emphysema in homozygous alpha1-antitrypsin-deficient persons.

In 9 alpha1-antitrypsin-deficient (PiZZ) persons 40 to 53 years of age, the relationships between the concentration of elastolytic protease of polymorphonuclear leukocyte granules and cigarette smoking, and the degree of abnormality in detailed pulmonary function tests were explored. Regardless of the statistical analytic approach used, elastase concentration was related more frequently and significantly than smoking history to the degree of abnormality observed in tests of pulmonary function. The data suggest that the severity of pulmonary dysfunction in PiZZ persons may be determined by variables other than age and cigarette smoking, including the concentration of elastolytic protease in leukocyte lysosomes.