Lung function, smoking, age and mortality data in 158 adult severe alpha
1-antitrypsin deficient, PiZZ individuals, followed from 1963 to 1982 were analyzed. Low initial FEV
1 value was significantly associated with increased mortality (
p < 0.005). A 3 yr mortality rate of 40% was found in individuals whose initial FEV
1 values were less than 30% of that predicted. In contrast, the corresponding 3-yr mortality among those whose initial FEV
1 values were between 30 and 65% of that predicted was only 7%. Smokers were found to have significantly lower FEV
1 levels (
p = 0.008) and higher mortality (
p < 0.005) than non-smokers. The difference between current and ex-smokers in mortality and FEV
1 level were not statistically significant (
p = 0.9 and
p>0.25, respectively). Cross-sectional analysis of the initial FEV
1 values indicated a significant decline (
p < 0.005) of FEV
1 with increasing age. This decline was greater among smokers than non-smokers. Longitudinal analysis of FEV
1 rates of decline in 80 cases with follow-up FEV
1 measurements failed to detect any significant differences between smokers and non-smokers, but was performed late in the disease process. The application of these results to the planning of studies on replacement therapy, smoking intervention strategy and longitudinal follow-up is discussed.
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