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. 2010 Apr 26;11(1):44.
doi: 10.1186/1465-9921-11-44.

Survival in severe alpha-1-antitrypsin deficiency (PiZZ)

Hanan A Tanash  1 , Peter M Nilsson, Jan-Ake Nilsson, Eeva Piitulainen
Affiliations

Survival in severe alpha-1-antitrypsin deficiency (PiZZ)

Hanan A Tanash et al. Respir Res. .

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).

Conclusion: Smokers with severe AAT deficiency, irrespective of mode of identification, have a significantly higher mortality risk than the general Swedish population.

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Figures

Figure 1
Figure 1
Kaplan-Meier analysis of mortality stratified by mode of identifiaction in 574 Respiratory cases, 431 Non-respiratory cases, and 334 Screened cases. The analysis showed a significant difference in survival time between R cases, NR cases and S cases, with estimated median survival times of 12 years, 14 years and 16 years, respectively (p < 0.001).
Figure 2
Figure 2
Kaplan-Meier analysis of mortality stratified by smoking habits in 737 smokers and 602 never-smokers. The analysis showed a significant difference in survival time between the smokers and the never-smokers, with estimated median survival times of 13 years and 14 years, respectively, (p < 0.01).
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References

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