title: Survival in severe alpha-1-antitrypsin deficiency (PiZZ).
abstract: BACKGROUND
  Previous studies of the natural history of alpha-1-antitrypsin (AAT) deficiency are mostly based on highly selected patients. The aim of this study was to analyse the mortality of PiZZ individuals.
  
  
  METHODS
  Data from 1339 adult PiZZ individuals from the Swedish National AAT Deficiency Registry, followed from 1991 to 2008, were analysed. Forty-three percent of these individuals were identified by respiratory symptoms (respiratory cases), 32% by liver diseases and other diseases (non-respiratory cases) and 25% by screening (screened cases). Smoking status was divided into two groups: smokers 737 (55%) and 602 (45%) never-smokers.
  
  
  RESULTS
  During the follow-up 315 individuals (24%) died. The standardised mortality rate (SMR) for respiratory cases was 4.70 (95% Confidence Interval (CI) 4.10-5.40), 3.0 (95%CI 2.35-3.70) for the non-respiratory cases and 2.30 (95% CI 1.46-3.46) for the screened cases. The smokers had a higher mortality risk than never-smokers, with a SMR of 4.80 (95%CI 4.20-5.50) for the smokers and 2.80(95%CI 2.30-3.40) for the never-smokers. The Rate Ratio (RR) was 1.70 (95% CI 1.35-2.20). Also among the screened cases, the mortality risk for the smokers was significantly higher than in the general Swedish population (SMR 3.40 (95% CI 1.98-5.40).
  
  
  CONCLUSIONS
  Smokers with severe AAT deficiency, irrespective of mode of identification, have a significantly higher mortality risk than the general Swedish population.
authors.affiliation: Department of Respiratory Medicine, Malmö University Hospital, Lund University, Malmö, 205 02, Sweden. Hanan.Tanash@med.lu.se
authors.forename: Hanan A
authors.initials: HA
authors.lastname: Tanash
authors.forename: Peter M
authors.initials: PM
authors.lastname: Nilsson
authors.forename: Jan-Ake
authors.initials: JA
authors.lastname: Nilsson
authors.forename: Eeva
authors.initials: E
authors.lastname: Piitulainen
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002423:Cause of Death; D018450:Disease Progression; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008168:Lung / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D016017:Odds Ratio; D016016:Proportional Hazards Models; D012042:Registries; D012129:Respiratory Function Tests; D018570:Risk Assessment; D012307:Risk Factors; D012720:Severity of Illness Index; D012907:Smoking / Q000401:mortality*; D013548:Sweden / Q000453:epidemiology; D013997:Time Factors; D055815:Young Adult; D019896:alpha 1-Antitrypsin Deficiency / Q000150:complications / Q000175:diagnosis / Q000401:mortality* / Q000503:physiopathology