Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2023 Nov;11(9):852-860.
doi: 10.1002/ueg2.12452. Epub 2023 Aug 25.

Four-fold increased mortality rate in patients with Wilson's disease: A population-based cohort study of 151 patients

Fredrik Åberg  1 , Ying Shang  2 , Rickard Strandberg  2 , Axel Wester  2 , Linnea Widman  2 , Hannes Hagström  2   3
Affiliations

Four-fold increased mortality rate in patients with Wilson's disease: A population-based cohort study of 151 patients

Fredrik Åberg et al. United European Gastroenterol J. 2023 Nov.

Abstract

Background and aims: Few studies have investigated mortality rates in patients with Wilson's disease and compared these to the general population. Here, we examined several clinical outcomes (including cardiovascular, psychiatric, neurologic conditions) in a population-based study of patients with Wilson's disease.

Method: We used nationwide registers to identify all patients with a first diagnosis of Wilson's disease between 2002 and 2020 in Sweden. Each patient was matched by age, sex, and municipality with up to 10 reference individuals from the general population. Validated registers were used to investigate outcomes up to 19 years after baseline in patients and reference individuals.

Results: We identified 151 patients with Wilson's disease matched with 1441 reference individuals. Median age at baseline was 26 years (IQR 17-42) and 50% were males. During a median follow-up of 6.6 years (IQR 2.9-12.9), 10 (6.6%) patients with Wilson's disease died compared with 31 (2.2%) reference individuals. This translated to a hazard ratio (HR) of 3.8 (95%CI = 1.8-8.1). Mortality was higher among Wilson's disease patients with baseline neuropsychiatric diagnoses (HR 7.9, 95%CI = 2.9-21.8). Cumulative mortality over 10 years was 9.3% (95%CI = 5.0-16.8) in Wilson's disease, compared to 2.4% (95%CI = 1.6-3.6) in reference individuals. We observed significantly elevated risks in the Wilson's disease group for incident cardiovascular disease, and incident psychiatric and neurological conditions when considering liver transplantation or death from other causes as competing events.

Conclusion: In this large population-based cohort study, patients with Wilson's disease had an almost four-fold increased mortality rate compared with matched individuals from the general population.

Keywords: OLT; Wilson; cardiovascular; death; liver transplantation; mortality; neurologic; neuropsychiatric; psychiatric.

PubMed Disclaimer

Conflict of interest statement

The authors declare no conflicts of interest.

Figures

FIGURE 1
FIGURE 1
Flowchart of inclusion and exclusion criteria.
FIGURE 2
FIGURE 2
Cumulative all‐cause mortality (panel A) among Wilson's disease patients and reference individuals, and the cumulative incidence of a composite endpoint of either death or liver transplantation (panel B).
FIGURE 3
FIGURE 3
The cumulative probability of liver transplantation and death without liver transplantation as competing‐risk events in a multistate analysis among Wilson's disease patients.
See this image and copyright information in PMC

Comment in

  • Challenges with neuropsychiatric Wilson's disease.
    Åberg F, Hagström H. Åberg F, et al. United European Gastroenterol J. 2023 Nov;11(9):906. doi: 10.1002/ueg2.12479. Epub 2023 Oct 18. United European Gastroenterol J. 2023. PMID: 37853947 Free PMC article. No abstract available.

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

References

    1. Członkowska A, Litwin T, Dusek P, Ferenci P, Lutsenko S, Medici V, et al. Wilson disease. Nat Rev Dis Primers. 2018;4(1):21. 10.1038/s41572-018-0018-3 - DOI - PMC - PubMed
    1. Huster D. Wilson disease. Best Pract Res Clin Gastroenterol. 2010;24(5):531–539. 10.1016/j.bpg.2010.07.014 - DOI - PubMed
    1. Beinhardt S, Leiss W, Stättermayer AF, Graziadei I, Zoller H, Stauber R, et al. Long‐term outcomes of patients with Wilson disease in a large Austrian cohort. Clin Gastroenterol Hepatol. 2014;12(4):683–689. 10.1016/j.cgh.2013.09.025 - DOI - PubMed
    1. Bruha R, Marecek Z, Pospisilova L, Nevsimalova S, Vitek L, Martasek P, et al. Long‐term follow‐up of Wilson Disease: natural history, treatment, mutations analysis and phenotypic correlation: long‐term follow‐up of Wilson disease. Liver Int. 2011;31(1):83–91. 10.1111/j.1478-3231.2010.02354.x - DOI - PubMed
    1. Członkowska A, Tarnacka B, Litwin T, Gajda J, Rodo M. Wilson’s disease—cause of mortality in 164 patients during 1992–2003 observation period. J Neurol. 2005;252(6):698–703. 10.1007/s00415-005-0720-4 - DOI - PubMed

Publication types