DECLINE IN FEV(1) AMONG PATIENTS WITH SEVERE HEREDITARY ALPHA(1)-ANTITRYPSIN DEFICIENCY TYPE PIZ

Severe alpha(1)-antitrypsin (alpha(1)-AT) deficiency is characterized by a decrease in serum alpha(1)-AT to values < 20% of normal. Severe a irflow obstruction, most commonly due to pulmonary emphysema secondary to the destruction of pulmonary elastic tissue, may develop at an ear ly age in persons with alpha(1)-AT deficiency. The purpose of this stu dy was to estimate the annual decline in FEV(1) (Delta FEV(1)) in alph a(1)-AT-deficient patients, to compare Delta FEV(1) in a referral popu lation (index cases) with subjects found through family studies (nonin dex cases), and to evaluate the role of smoking cessation on Delta FEV (1) . One hundred and sixty-one subjects from the Danish alpha(1)-AT-d eficiency study who were older than 25 yr and who had a recorded smoki ng history and at least two spirometric examinations 1 yr apart were s tudied. The Delta FEV(1) for each individual was determined by regress ion analysis of FEV(1) on follow-up time. The overall mean Delta FEV(1 ) was 81 ml/yr. There was no significant difference in Delta FEV(1) be tween 113 index cases and 48 nonindex cases even after controlling for age, initial FEV(1), sex, and lifetime tobacco consumption. One hundr ed of the subjects were ex-smokers, 18 had never smoked, and 43 were c urrent smokers. The mean Delta FEV(1) among the current smokers was 13 2 ml/yr, versus 52 ml/yr among the ex-smokers (p < 0.001). For the nev er-smokers, the mean Delta FEV(1) was 86 ml/yr.