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Cancer risk in severe alpha-1-antitrypsin deficiency: the importance of early identification

Hiller, Adriana Maria LU orcid ; Ekström, Magnus LU orcid ; Piitulainen, Eeva LU ; Lindberg, Anne ; Rönmark, Eva and Tanash, Hanan LU (2022) In European Respiratory Journal 60(5).
Abstract

Background Severe alpha-1-antitrypsin deficiency (AATD), phenotype PiZZ, is a risk factor for pulmonary emphysema and liver disease, but its effect on cancer risk is unknown. Our aim was to evaluate the risk and the risk factors for incident cancer in PiZZ individuals compared with the general population with known smoking habits. Methods A longitudinal study of PiZZ individuals (n=1,595) from the Swedish National AATD Register, and controls (n=5,999) from Swedish population-based cohorts. Data on cancer and mortality were obtained by cross-linkage with national registers. Individuals who had undergone lung transplantation (n=10) and those with a cancer diagnosis within five years prior to inclusion (n=63) were excluded. The risk... (More)

Background Severe alpha-1-antitrypsin deficiency (AATD), phenotype PiZZ, is a risk factor for pulmonary emphysema and liver disease, but its effect on cancer risk is unknown. Our aim was to evaluate the risk and the risk factors for incident cancer in PiZZ individuals compared with the general population with known smoking habits. Methods A longitudinal study of PiZZ individuals (n=1,595) from the Swedish National AATD Register, and controls (n=5,999) from Swedish population-based cohorts. Data on cancer and mortality were obtained by cross-linkage with national registers. Individuals who had undergone lung transplantation (n=10) and those with a cancer diagnosis within five years prior to inclusion (n=63) were excluded. The risk factors for developing cancer were analyzed using proportional hazards and Fine-Gray regression models, adjusting for age, sex, smoking habits and the presence of liver disease. Results The median follow-up time was 17 years (IQR 11) for the whole study population. The incidence rate of hepatic and non-hepatic cancer per 1,000 person-years was 1.6 (95% CI 1.1-2.3) and 8.5 (95% CI 7.2-10.0) for the PiZZ individuals, and 0.1 (95% CI 0.04-0.2) and 6.6 (95% CI 6.0-7.1) for the controls, respectively. The adjusted hazard ratios (HR) for hepatic and for non-hepatic cancer were 23.4 (95% CI 9.9-55.4) and 1.3 (95% CI 1.1-1.5) respectively, in the PiZZ individuals compared with the controls. Conclusion These results suggest that individuals with severe alpha-1-antitrypsin deficiency may have an increased risk of developing both hepatic and non-hepatic cancer, compared with the general population.

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author
; ; ; ; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Alpha-1-antitrypsin deficiency, cancer, hepatic cancer, risk factors, survival
in
European Respiratory Journal
volume
60
issue
5
article number
2200846
publisher
European Respiratory Society
external identifiers
  • pmid:35361631
  • scopus:85141646715
ISSN
0903-1936
DOI
10.1183/13993003.00846-2022
language
English
LU publication?
yes
id
678d73bc-35b1-4154-b2a1-aaea42331490
date added to LUP
2022-12-01 14:47:04
date last changed
2024-02-17 19:40:20
@article{678d73bc-35b1-4154-b2a1-aaea42331490,
  abstract     = {{<p>Background Severe alpha-1-antitrypsin deficiency (AATD), phenotype PiZZ, is a risk factor for pulmonary emphysema and liver disease, but its effect on cancer risk is unknown. Our aim was to evaluate the risk and the risk factors for incident cancer in PiZZ individuals compared with the general population with known smoking habits. Methods A longitudinal study of PiZZ individuals (n=1,595) from the Swedish National AATD Register, and controls (n=5,999) from Swedish population-based cohorts. Data on cancer and mortality were obtained by cross-linkage with national registers. Individuals who had undergone lung transplantation (n=10) and those with a cancer diagnosis within five years prior to inclusion (n=63) were excluded. The risk factors for developing cancer were analyzed using proportional hazards and Fine-Gray regression models, adjusting for age, sex, smoking habits and the presence of liver disease. Results The median follow-up time was 17 years (IQR 11) for the whole study population. The incidence rate of hepatic and non-hepatic cancer per 1,000 person-years was 1.6 (95% CI 1.1-2.3) and 8.5 (95% CI 7.2-10.0) for the PiZZ individuals, and 0.1 (95% CI 0.04-0.2) and 6.6 (95% CI 6.0-7.1) for the controls, respectively. The adjusted hazard ratios (HR) for hepatic and for non-hepatic cancer were 23.4 (95% CI 9.9-55.4) and 1.3 (95% CI 1.1-1.5) respectively, in the PiZZ individuals compared with the controls. Conclusion These results suggest that individuals with severe alpha-1-antitrypsin deficiency may have an increased risk of developing both hepatic and non-hepatic cancer, compared with the general population.</p>}},
  author       = {{Hiller, Adriana Maria and Ekström, Magnus and Piitulainen, Eeva and Lindberg, Anne and Rönmark, Eva and Tanash, Hanan}},
  issn         = {{0903-1936}},
  keywords     = {{Alpha-1-antitrypsin deficiency; cancer; hepatic cancer; risk factors; survival}},
  language     = {{eng}},
  month        = {{11}},
  number       = {{5}},
  publisher    = {{European Respiratory Society}},
  series       = {{European Respiratory Journal}},
  title        = {{Cancer risk in severe alpha-1-antitrypsin deficiency: the importance of early identification}},
  url          = {{http://dx.doi.org/10.1183/13993003.00846-2022}},
  doi          = {{10.1183/13993003.00846-2022}},
  volume       = {{60}},
  year         = {{2022}},
}