Advanced

Cause-specific mortality in individuals with severe alpha 1-antitrypsin deficiency in comparison with the general population in Sweden

Tanash, Hanan A. LU ; Ekström, Magnus LU ; Wagner, Philippe and Piitulainen, Eeva LU (2016) In International Journal of COPD 11(1). p.1663-1669
Abstract

Background: Severe alpha 1-antitrypsin deficiency (PiZZ) predisposes to morbidity and mortality due to early-onset emphysema and liver disease. The risk of death from other causes, including cardiovascular disease and cancer, has not been well investigated. We aimed to analyze cause-specific mortality in PiZZ individuals compared with the general Swedish population. Methods: Data on 1,561 PiZZ individuals from the Swedish National AAT Deficiency Register, prospectively followed from 1991 to 2014, were analyzed. Causes of death according to the Swedish National Causes of Death Register for the study group were compared with those for the general Swedish population matched for age, sex, and calendar year, with the excess mortality... (More)

Background: Severe alpha 1-antitrypsin deficiency (PiZZ) predisposes to morbidity and mortality due to early-onset emphysema and liver disease. The risk of death from other causes, including cardiovascular disease and cancer, has not been well investigated. We aimed to analyze cause-specific mortality in PiZZ individuals compared with the general Swedish population. Methods: Data on 1,561 PiZZ individuals from the Swedish National AAT Deficiency Register, prospectively followed from 1991 to 2014, were analyzed. Causes of death according to the Swedish National Causes of Death Register for the study group were compared with those for the general Swedish population matched for age, sex, and calendar year, with the excess mortality expressed as standardized mortality ratios (SMRs) with 95% confidence intervals (CIs). Results: There were 524 deaths during the follow-up period. PiZZ individuals had excess all-cause mortality compared with the Swedish general population (SMR 3.6, 95% CI 3.3–3.9). SMR for ischemic heart disease (IHD) was 0.5 (95% CI 0.3–0.8) and was similar for never and ever-smokers, and in males and females. SMR for lung cancer was 0.9 (95% CI 0.4–1.7). PiZZ individuals had increased mortality compared with the general population for the following diseases: respiratory disease, SMR 48.4 (95% CI 43.0–54.5); primary liver carcinoma, SMR 90.0 (95% CI 59.3–130.9); complicated colon diverticulitis, SMR 20.8 (95% CI 6.7–48.6); and pulmonary embolism, SMR 6.9 (95% CI 3.3–12.7). Conclusion: PiZZ individuals had a reduced mortality risk of IHD. Mortality due to respiratory, hepatic disease, diverticulitis, and pulmonary embolism was markedly increased compared with the age- and sex-matched Swedish population.

(Less)
Please use this url to cite or link to this publication:
author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Alpha 1-antitrypsin deficiency, Cause-specific mortality, Ischemic heart disease, Pulmonary embolism, Standardized mortality ratio
in
International Journal of COPD
volume
11
issue
1
pages
7 pages
publisher
Dove Press
external identifiers
  • Scopus:84983269101
ISSN
1176-9106
DOI
10.2147/COPD.S109173
language
English
LU publication?
yes
id
a2c216e9-ff38-44c2-93b1-6dfca4f342ce
date added to LUP
2016-10-04 08:44:10
date last changed
2016-10-20 10:59:31
@misc{a2c216e9-ff38-44c2-93b1-6dfca4f342ce,
  abstract     = {<p>Background: Severe alpha 1-antitrypsin deficiency (PiZZ) predisposes to morbidity and mortality due to early-onset emphysema and liver disease. The risk of death from other causes, including cardiovascular disease and cancer, has not been well investigated. We aimed to analyze cause-specific mortality in PiZZ individuals compared with the general Swedish population. Methods: Data on 1,561 PiZZ individuals from the Swedish National AAT Deficiency Register, prospectively followed from 1991 to 2014, were analyzed. Causes of death according to the Swedish National Causes of Death Register for the study group were compared with those for the general Swedish population matched for age, sex, and calendar year, with the excess mortality expressed as standardized mortality ratios (SMRs) with 95% confidence intervals (CIs). Results: There were 524 deaths during the follow-up period. PiZZ individuals had excess all-cause mortality compared with the Swedish general population (SMR 3.6, 95% CI 3.3–3.9). SMR for ischemic heart disease (IHD) was 0.5 (95% CI 0.3–0.8) and was similar for never and ever-smokers, and in males and females. SMR for lung cancer was 0.9 (95% CI 0.4–1.7). PiZZ individuals had increased mortality compared with the general population for the following diseases: respiratory disease, SMR 48.4 (95% CI 43.0–54.5); primary liver carcinoma, SMR 90.0 (95% CI 59.3–130.9); complicated colon diverticulitis, SMR 20.8 (95% CI 6.7–48.6); and pulmonary embolism, SMR 6.9 (95% CI 3.3–12.7). Conclusion: PiZZ individuals had a reduced mortality risk of IHD. Mortality due to respiratory, hepatic disease, diverticulitis, and pulmonary embolism was markedly increased compared with the age- and sex-matched Swedish population.</p>},
  author       = {Tanash, Hanan A. and Ekström, Magnus and Wagner, Philippe and Piitulainen, Eeva},
  issn         = {1176-9106},
  keyword      = {Alpha 1-antitrypsin deficiency,Cause-specific mortality,Ischemic heart disease,Pulmonary embolism,Standardized mortality ratio},
  language     = {eng},
  month        = {07},
  number       = {1},
  pages        = {1663--1669},
  publisher    = {ARRAY(0xb821ca0)},
  series       = {International Journal of COPD},
  title        = {Cause-specific mortality in individuals with severe alpha 1-antitrypsin deficiency in comparison with the general population in Sweden},
  url          = {http://dx.doi.org/10.2147/COPD.S109173},
  volume       = {11},
  year         = {2016},
}