title: Survival benefit of lung transplantation in individuals with severe α1-anti-trypsin deficiency (PiZZ) and emphysema.
abstract: BACKGROUND
  The objective of lung transplantation (LTx) is to prolong life, but the survival benefit for patients with severe α(1)-anti-trypsin deficiency (PiZZ) and emphysema is still unclear. The aim of this study was to assess whether PiZZ patients who have undergone lung transplantation (the lung transplant group, TxG) do better than patients who have continued on the usual medical therapy (the non-transplant group, NTxG).
  
  
  METHODS
  Between 1990, when the first patient received a lung transplant in Sweden, until June 2010, a total of 83 PiZZ patients with severe emphysema underwent transplantation. Seventy appropriate controls were identified from the Swedish National AAT Deficiency Registry. Each control was matched with a patient who had received a lung transplant, for age, gender, smoking history (number of pack-years) and lung function at the time of transplantation.
  
  
  RESULTS
  Both controls and lung transplant patients had low spirometric values with a mean FEV(1) of 23 ± 6% and 22 ± 9% of predicted value, respectively (not a statistically significant difference). Of the 83 transplant patients, 62 (75%) underwent single-lung transplantation (SLTx). During follow-up, 37 (45%) deaths occurred in the TxG and 45 (64%) in the NTxG. In the TxG, the estimated median survival time was 11 years (95% confidence interval [CI] 9 to 14 years), compared with 5 years (95% CI 4 to 6 years) for the NTxG (p = 0.006). The most common cause of death was pulmonary infection among the transplant patients (38%) and respiratory failure (60%) among the controls.
  
  
  CONCLUSIONS
  Lung transplantation significantly improves long-term survival of patients with severe α(1)-anti-trypsin deficiency (PiZZ) and emphysema.
authors.affiliation: Department of Respiratory Medicine, Skåne University Hospital, Lund University, Lund, Sweden. hanan.tanash@med.lu.se
authors.forename: Hanan A
authors.initials: HA
authors.lastname: Tanash
authors.forename: Gerdt C
authors.initials: GC
authors.lastname: Riise
authors.forename: Lennart
authors.initials: L
authors.lastname: Hansson
authors.forename: Peter M
authors.initials: PM
authors.lastname: Nilsson
authors.forename: Eeva
authors.initials: E
authors.lastname: Piitulainen
mesh_terms: D000328:Adult; D000368:Aged; D016022:Case-Control Studies; D015897:Comorbidity; D004646:Emphysema / Q000150:complications / Q000453:epidemiology* / Q000601:surgery*; D005260:Female; D006801:Humans; D015994:Incidence; D016040:Lung Transplantation; D008297:Male; D008875:Middle Aged; D012042:Registries; D012131:Respiratory Insufficiency / Q000453:epidemiology / Q000209:etiology; D012141:Respiratory Tract Infections / Q000453:epidemiology / Q000209:etiology; D012189:Retrospective Studies; D012720:Severity of Illness Index; D013147:Spirometry; D015996:Survival Rate; D013548:Sweden; D016896:Treatment Outcome; D019896:alpha 1-Antitrypsin Deficiency / Q000150:complications / Q000453:epidemiology* / Q000601:surgery*