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Thorax 2008;63:1091-1095 doi:10.1136/thx.2008.095497
  • Alpha-1-antitrypsin deficiency

Clinical course and prognosis of never-smokers with severe alpha-1-antitrypsin deficiency (PiZZ)

  1. H A Tanash1,
  2. P M Nilsson2,
  3. J-Å Nilsson1,
  4. E Piitulainen1
  1. 1
    Department of Respiratory Medicine, Malmö University Hospital, Lund University, Sweden
  2. 2
    Department of Clinical Sciences, Malmö University Hospital, Lund University, Sweden
  1. Dr H Tanash, Department of Respiratory Medicine, Malmö University Hospital, Entrance 35, S-205 02, Malmö, Sweden; hanan.tanash{at}med.lu.se
  • Received 12 January 2008
  • Accepted 15 June 2008
  • Published Online First 5 August 2008

Abstract

Background: Previous studies of non-smoking individuals with severe α1-antitrypsin deficiency (PiZZ) have been sparse and included only a limited number of individuals, mostly identified by respiratory symptoms. The aim of this study was to estimate the prognosis of non-smoking PiZZ individuals and to analyse the most common causes of death by including a large number of individuals who had been identified by other means than respiratory symptoms.

Methods: The study included 568 non-smoking PiZZ subjects who were selected from the Swedish National AAT Deficiency Registry and followed up from 1991 to September 2007. Of these, 156 (27%) were identified by respiratory symptoms (respiratory cases) and 412 were identified by extrapulmonary symptoms or screening (non-respiratory cases).

Results: 93 subjects (16%) died during the follow-up period. The specific standardised mortality rate (SMR) for the whole study population was 2.32 (95% CI 1.87 to 2.83) with no significant difference between men and women. The SMR was 2.55 (95% CI 1.91 to 2.83) for the respiratory cases and 2.07 (95% CI 1.49 to 2.81) for the non-respiratory cases. Further calculation of SMR for subgroups in the non-respiratory cases showed that the SMR was 0.70 (95% CI 0.14 to 2.04) for individuals identified by family/population screening. Emphysema and liver cirrhosis were the most common causes of death (45% and 28%, respectively). Malignant transformation was found in 38% of the cases with cirrhosis.

Conclusion: Non-smoking PiZZ individuals identified by screening do not have an increased mortality risk compared with the Swedish general population.

Footnotes

  • Funding: This work was supported by grants from the Swedish National Heart-Lung Foundation.

  • Competing interests: None.

  • Ethics approval: The Swedish AAT Registry is approved by the local research and ethics committee (Institutional Ethics Committee, Lund University, Sweden) and by the Swedish Data Inspection Board. Written informed consent was obtained from each subject prior to inclusion in the register.

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