Journal
EUROPEAN RESPIRATORY JOURNAL
Volume 50, Issue 3, Pages -Publisher
EUROPEAN RESPIRATORY SOC JOURNALS LTD
DOI: 10.1183/13993003.00198-2017
Keywords
-
Categories
Funding
- Swedish Heart-Lung Foundation
- Swedish Society of Medicine and the Swedish Heart-Lung Foundation
Ask authors/readers for more resources
Knowledge about the natural history of severe alpha 1-antitrypsin (AAT) deficiency (PiZZ) is limited. Our aim was to compare the survival of PiZZ individuals with randomly selected controls from the Swedish general population. The PiZZ subjects (n= 1585) were selected from the Swedish National AATD Register. The controls (n= 5999) were randomly selected from the Swedish population register. Smoking habits were known for all subjects. Median follow-up times for the PiZZ subjects (731 never-smokers) and controls (3179 never-smokers) were 12 and 17 years, respectively (p<0.001). During follow-up, 473 PiZZ subjects (30%), and 747 controls (12%) died. The PiZZ subjects had a significantly shorter survival time than the controls, p<0.001. After adjustment for gender, age, smoking habits and presence of respiratory symptoms, the risk of death was still significantly higher for the PiZZ individuals than for the controls, hazard ratio (HR) 3.2 (95% CI 2.83.6; p<0.001). By contrast, the risk of death was not increased in never-smoking PiZZ individuals identified by screening, compared to never-smoking controls, HR 1.2 (95% CI 0.6-2.2). The never-smoking PiZZ individuals identified by screening had a similar life expectancy to the neversmokers in the Swedish general population. Early diagnosis of AAT deficiency is of utmost importance.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available