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Cited by 242Adrenal Hormones and DisordersImmunodeficiency and Autoimmune DisordersPituitary Gland Disorders and Treatments

Increased death risk and altered cancer incidence pattern in patients with isolated or combined autoimmune primary adrenocortical insufficiency

Bensing, Sophie, Brandt, Lena, Tabaroj, Farnoush, Sjöberg, Olof, Nilsson, Bo, Ekbom, Anders, Blomqvist, Paul, Kämpe, Olle·2008·Clinical Endocrinology
DOI 10.1111/j.1365-2265.2008.03340.x
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Abstract

Objectives Primary adrenocortical insufficiency is mostly caused by an autoimmune destruction of the adrenal cortex. The disease may appear isolated or as a part of an autoimmune polyendocrine syndrome (APS). APS1 is a rare hereditary disorder with a broad spectrum of clinical manifestations. In APS2, primary adrenocortical insufficiency is often combined with autoimmune thyroid disease and/or type 1 diabetes. We analysed mortality and cancer incidence in primary adrenocortical insufficiency patients during 40 years. Data were compared with the general Swedish population. Design and patients A population based cohort study including all patients with autoimmune primary adrenocortical insufficiency (3299) admitted to Swedish hospitals 1964-2004. Measurements Mortality risk was calculated as the standardized mortality ratio (SMR) and cancer incidence as the standardized incidence ratio (SIR). Results A more than 2-fold increased mortality risk was observed in both women (SMR 2.9, 95% CI 2.7-3.0) and men (SMR 2.5, 95% CI 2.3-2.7). Highest risks were observed in patients diagnosed in childhood. SMR was higher in APS1 patients (SMR 4.6, 95% CI 3.5-6.0) compared with patients with APS2 (SMR 2.1, 95% CI 1.9-2.4). Cancer incidence was increased (SIR 1.3, 95% CI 1.2-1.5). When tumours observed during the first year of follow-up were excluded, only the cancer risk among APS1 patients remained increased. Cause-specific cancer incidence analysis revealed significantly higher incidences o…

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Authors
Bensing, S., Brandt, L., Tabaroj, F., Sjöberg, O., Nilsson, B., Ekbom, A., Blomqvist, P., & Kämpe, O.
Affiliations
  • Uppsala University (SE)
  • Karolinska University Hospital (SE)
  • Uppsala University Hospital (SE)
Corresponding author
Bensing, Sophie
ORCID
Venue
Clinical Endocrinology
Publisher
Wiley
Published
2008-10-14
Volume
69
Issue
5
Pages
697-704
ISSN
  • 0300-0664 (ISSN-L)
  • 1365-2265
DOI
10.1111/j.1365-2265.2008.03340.x
OpenAlex
W2097490026
Cited by
242
References
0
Citations by year
2026: 3 · 2025: 6 · 2024: 10 · 2023: 10 · 2022: 10 · 2021: 25 · 2020: 18 · 2019: 16 · 2018: 15 · 2017: 17 · 2016: 21 · 2015: 12 · 2014: 26 · 2013: 15 · 2012: 10
FWCI
6.76
Type
journal article
Language
en
Open access
closed
Licence
onlinelibrary.wiley.com/termsAndConditions#vor (vor)
Locations
  • Clinical Endocrinology (publishedVersion)
  • PubMed (publishedVersion)
Funders
Vetenskapsrådet
Topics
  • Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Adrenal Hormones and Disorders
  • Life Sciences › Immunology and Microbiology › Immunology › Immunodeficiency and Autoimmune Disorders
  • Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Pituitary Gland Disorders and Treatments
Keywords
autoimmune polyendocrine syndrome type 1, autoimmune polyendocrine syndrome type 2, mortality risk, cancer incidence, oral cancer, non-melanoma skin cancer, breast cancer, adrenal insufficiency, life expectancy
MeSH terms
Addison Disease, Adolescent, Adult, Aged, Aged, 80 and over, Autoimmune Diseases, Child, Child, Preschool, Diabetes Mellitus, Type 1, Female, Humans, Infant, Infant, Newborn, Male, Middle Aged, Neoplasms, Risk Factors, Sweden, Thyroid Diseases, Cohort Studies, Comorbidity, Incidence, Young Adult

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