title: Prognosis of patients with alpha1-antitrypsine deficiency on long-term oxygen therapy.
abstract: BACKGROUND
  Data on patients with alpha1-antitrypsine deficiency (AATD) on long-term oxygen therapy (LTOT) is sparse. The aim of this study was to present the incidence of patients with AATD on LTOT, and compare their characteristics, comorbidities and prognosis (lung transplantation, termination of LTOT, and survival) with COPD patients without AATD.
  
  
  METHODS
  A National prospective study of all COPD patients who started LTOT for the first time in the period 01.11.1994 to 31.12.2010.
  
  
  RESULTS
  Among the 21,964 patients on LTOT, 234 patients had AATD. AATD patients were more often males and were on average about 17 years younger than patients without AATD. Cardio-vascular diseases and diabetes mellitus were significantly less prevalent among patients with AATD (60.4% versus 70.3% (P < 0.001) and 4.7% versus 12.2% (P < 0.001)), whereas osteoporosis was more frequent (28.5% versus 20.4%, p = 0.002. Eighty-nine (38.0%) AATD patients and 173 (0.8%) non-AATD patients were lung transplanted in the study period. Median survival was 8.7 years in AATD patients with lung transplantation, 3.3 years in AATD patients without lung transplantation, 6.3 years in non-AATD patients with lung transplantation, and 1.6 years in non-AATD without lung transplantation. Even after adjustment for gender, age, comorbidities, and the time between start of LTOT and lung transplantation, patients with AATD had a lower risk of death compared to non-AATD patients (Hazard ratio 0.73 (95% CI: 0.62-0.86; P < 0.001).
  
  
  CONCLUSIONS
  Compared with COPD without AATD, AATD patients are younger, more often males, have a lower prevalence of cardio-vascular diseases and diabetes mellitus, and higher prevalence of osteoporosis. Moreover, they have better prognosis, partly due to greater chance of receiving a lung transplantation.
authors.affiliation: Department of Respiratory Medicine, University Hospital of Copenhagen, Hvidovre Hospital, Denmark. Electronic address: ringbaek@dadlnet.dk.
authors.forename: Thomas J
authors.initials: TJ
authors.lastname: Ringbaek
authors.affiliation: Department of Respiratory Medicine, University Hospital of Copenhagen, Gentofte Hospital, Denmark.
authors.forename: Niels
authors.initials: N
authors.lastname: Seersholm
authors.affiliation: Department of Cardiology, Section of Lung Transplantation, Copenhagen University Hospital, Rigshospitalet, Denmark.
authors.forename: Michael
authors.initials: M
authors.lastname: Perch
authors.affiliation: Department of Cardiology, Section of Lung Transplantation, Copenhagen University Hospital, Rigshospitalet, Denmark.
authors.forename: Martin
authors.initials: M
authors.lastname: Iversen
authors.affiliation: Department of Respiratory Medicine, University Hospital of Copenhagen, Hvidovre Hospital, Denmark; Department of Public Health, Section of Social Medicine, University of Copenhagen, Denmark.
authors.forename: Peter
authors.initials: P
authors.lastname: Lange
mesh_terms: D002318:Cardiovascular Diseases / Q000150:complications / Q000401:mortality; D048909:Diabetes Complications / Q000150:complications / Q000401:mortality; D005260:Female; D006801:Humans; D008134:Long-Term Care; D016040:Lung Transplantation / Q000401:mortality; D008297:Male; D008875:Middle Aged; D010024:Osteoporosis / Q000150:complications / Q000401:mortality; D010100:Oxygen / Q000627:therapeutic use*; D011379:Prognosis; D011446:Prospective Studies; D029424:Pulmonary Disease, Chronic Obstructive / Q000150:complications / Q000401:mortality / Q000628:therapy*; D012737:Sex Factors; D019896:alpha 1-Antitrypsin Deficiency / Q000150:complications* / Q000401:mortality
chemical_list: D010100:Oxygen
keywords: Alpha(1)-antitrypsine deficiency; COPD; LTOT; Oxygen therapy; Survival