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Cancer risk in severe alpha-1-antitrypsin deficiency

Adriana-Maria Hiller, Magnus Ekström, Eeva Piitulainen, Anne Lindberg, Eva Rönmark, Hanan Tanash
European Respiratory Journal 2022 60: 2103200; DOI: 10.1183/13993003.03200-2021
Adriana-Maria Hiller
1Dept of Respiratory Medicine and Allergology, Lund University, Skåne University Hospital, Malmö, Sweden
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  • ORCID record for Adriana-Maria Hiller
  • For correspondence: adriana-maria.hiller@med.lu.se
Magnus Ekström
2Lund University, Faculty of Medicine, Dept of Clinical Sciences Lund, Respiratory Medicine and Allergology, Lund, Sweden
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Eeva Piitulainen
1Dept of Respiratory Medicine and Allergology, Lund University, Skåne University Hospital, Malmö, Sweden
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Anne Lindberg
3Dept of Public Health and Clinical Medicine, Section of Medicine, the OLIN unit, Umeå University, Umeå, Sweden
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Eva Rönmark
4Dept of Public Health and Clinical Medicine, Section of Sustainable Health, the OLIN unit, Umeå University, Umeå, Sweden
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Hanan Tanash
1Dept of Respiratory Medicine and Allergology, Lund University, Skåne University Hospital, Malmö, Sweden
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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=1595) from the Swedish National AATD Register, and controls (n=5999) 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 5 years prior to inclusion (n=63) were excluded. The risk factors for developing cancer were analysed 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 (interquartile range 11 years) for the whole study population. The incidence rates of hepatic and non-hepatic cancer per 1000 person-years were 1.6 (95% CI 1.1–2.3) and 8.5 (95% CI 7.2–10.0), respectively, for the PiZZ individuals, and 0.1 (95% CI 0.04–0.2) and 6.6 (95% CI 6.0–7.1), respectively, for the controls. The adjusted hazard ratios 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 AATD may have an increased risk of developing both hepatic and non-hepatic cancer, compared with the general population.

Abstract

Individuals with severe alpha-1-antitrypsin deficiency appear to have an increased risk of developing cancer compared with the general population, even after adjustment for age, sex, smoking habits and liver disease. https://bit.ly/3ikSy0W

Footnotes

  • This article has an editorial commentary: https://doi.org/10.1183/13993003.01289-2022

  • Author contributions: All authors have made substantial contributions to the concept and design of the study, acquisition of the data, or the analysis and interpretation of the data. All took part in drafting the article and revising it critically for important intellectual content, gave their final approval of the version to be published, and agreed to be accountable for all aspects of the work.

  • Conflict of interest: A. Lindberg reports lecture honoraria from Boehringer Ingelheim and Novartis; personal fees for advisory board participation with AstraZeneca, GlaxoSmithKline, Novartis and Boehringer Ingelheim; outside the submitted work. All other authors have nothing to disclose.

  • Support statement: H. Tanash was supported by unrestricted grants from the Swedish Heart-Lung Foundation, the Skåne University Hospital and the Swedish Society of Medicine. E. Piitulainen was supported by unrestricted grants from the Swedish Heart-Lung Foundation. M. Ekström was supported by unrestricted grants from the Swedish Society of Medicine and the Swedish Research Council (Dnr 2019-02081). A. Lindberg and E. Rönmark were supported by unrestricted grants from the Norrbotten County Council. The recruitment of the OLIN cohorts was supported mainly by the Swedish Heart-Lung Foundation, the Swedish Asthma-Allergy Foundation, and ALF, a regional agreement between Umeå University and Norrbotten County Council.

  • Received December 19, 2021.
  • Accepted March 12, 2022.
  • Copyright ©The authors 2022. For reproduction rights and permissions contact permissions{at}ersnet.org
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Cancer risk in severe alpha-1-antitrypsin deficiency
Adriana-Maria Hiller, Magnus Ekström, Eeva Piitulainen, Anne Lindberg, Eva Rönmark, Hanan Tanash
European Respiratory Journal Oct 2022, 60 (4) 2103200; DOI: 10.1183/13993003.03200-2021

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Cancer risk in severe alpha-1-antitrypsin deficiency
Adriana-Maria Hiller, Magnus Ekström, Eeva Piitulainen, Anne Lindberg, Eva Rönmark, Hanan Tanash
European Respiratory Journal Oct 2022, 60 (4) 2103200; DOI: 10.1183/13993003.03200-2021
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