Occupational exposure risks in individuals with PI*Z alpha(1)-antitrypsin deficiency

We obtained questionnaire and spirometry data from 128 alpha(1)-antitrypsin (alpha(1)AT)-deficient individuals with phenotype PI*Z to examine the rela tionship between chronic respiratory symptoms, airflow limitation, treatmen t requirements, and semiquantitative estimates of occupational exposure to dust, fumes, smoke, and gas. After adjusting for age, smoking, and prior lo wer respiratory tract infections, increased prevalence of chronic cough (OR = 4.69, 95% Cl = 1.57-13.74, p = 0.006) and having left a job due to breat hlessness (OR = 2.72 95% Cl = 1.07-6.92, p = 0.036) were seen in individual s reporting high mineral dust exposure compared with those with no exposure . Subjects reporting high mineral dust exposure also had significantly lowe r FEV1 (31% predicted for high exposure versus 36% for low and 40% for unex posed, p = 0.032). The excess risk of chronic cough seen with occupational fumes or smoke exposure disappeared after adjusting for mineral dust exposu re, but the association with lower FEV1/FVC ratio persisted (p = 0.022). Pe rsonal tobacco use was a significant risk factor for most outcome measures, but no interaction with occupational exposure was seen. These results sugg est that occupational inhalational exposures are independently associated w ith respiratory symptoms and airflow limitation in severely alpha(1)AT-defi cient individuals.