PT  - JOURNAL ARTICLE
AU  - Hanan A. Tanash
AU  - Magnus Ekström
AU  - Eva Rönmark
AU  - Anne Lindberg
AU  - Eeva Piitulainen
TI  - Survival in individuals with severe alpha 1-antitrypsin deficiency (PiZZ) in comparison to a general population with known smoking habits
AID  - 10.1183/13993003.00198-2017
DP  - 2017 Sep 01
TA  - European Respiratory Journal
PG  - 1700198
VI  - 50
IP  - 3
4099  - http://erj.ersjournals.com/content/50/3/1700198.short
4100  - http://erj.ersjournals.com/content/50/3/1700198.full
SO  - Eur Respir J2017 Sep 01; 50
AB  - 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 2.8–3.6; 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 never-smokers in the Swedish general population. Early diagnosis of AAT deficiency is of utmost importance.Early diagnosis of AAT deficiency is of utmost importance http://ow.ly/5iHm30dKYqN