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Cited by 758Adrenal Hormones and DisordersImmunodeficiency and Autoimmune DisordersMyasthenia Gravis and Thymoma

Autoimmune Adrenal Insufficiency and Autoimmune Polyendocrine Syndromes: Autoantibodies, Autoantigens, and Their Applicability in Diagnosis and Disease Prediction

Betterle, Corrado, Dal Pra, Chiara, Mantero, Franco, Zanchetta, Renato·2002·Endocrine Reviews
DOI 10.1210/edrv.23.3.0466
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Abstract

Recent progress in the understanding of autoimmune adrenal disease, including a detailed analysis of a group of patients with Addison's disease (AD), has been reviewed. Criteria for defining an autoimmune disease and the main features of autoimmune AD (history, prevalence, etiology, histopathology, clinical and laboratory findings, cell-mediated andhumoral immunity, autoantigens and their autoepitopes, genetics, animal models, associated autoimmune diseases, pathogenesis, natural history, therapy) have been described. Furthermore, the autoimmune polyglandular syndromes (APS) associated with AD (revised classification, animal models, genetics, natural history) have been discussed. Of Italian patients with primary AD (n = 317), 83% had autoimmune AD. At the onset, all patients with autoimmune AD (100%) had detectable adrenal cortex and/or steroid 21-hydroxylase autoantibodies. In the course of natural history of autoimmune AD, the presence of adrenal cortex and/or steroid 21-hydroxylase autoantibodies identified patients at risk to develop AD. Different risks of progression to clinical AD were found in children and adults, and three stages of subclinical hypoadrenalism have been defined. Normal or atrophic adrenal glands have been demonstrated by imaging in patients with clinical or subclinical AD. Autoimmune AD presented in four forms: as APS type 1 (13% of the patients), APS type 2 (41%), APS type 4 (5%), and isolated AD (41%). There were differences in genetics, age at onset…

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  • The risk of developing Addison disease is 10 times higher in patients with type 1 diabetes mellitus than in the general population.

    “Since the risk of developing AD is 10 times higher in patients with T1DM than in the general population, and the combination of concomitant T1DM and AD is associated with a more than 4-fold increase in the risk of premature death compared with patients with T1DM alone, awareness of this risk is of paramount importance to physicians managing patients with T1DM [6, 7].”

    From Onset of Addison Disease in a Patient Using the Advanced Hybrid Closed-Loop MiniMed 780G: Diagnostic Challenges (2025) · cites this paper as [7]

  • The combination of concomitant type 1 diabetes mellitus and Addison disease is associated with a more than 4-fold increase in the risk of premature death compared with patients with type 1 diabetes mellitus alone.

    “Since the risk of developing AD is 10 times higher in patients with T1DM than in the general population, and the combination of concomitant T1DM and AD is associated with a more than 4-fold increase in the risk of premature death compared with patients with T1DM alone, awareness of this risk is of paramount importance to physicians managing patients with T1DM [6, 7].”

    From Onset of Addison Disease in a Patient Using the Advanced Hybrid Closed-Loop MiniMed 780G: Diagnostic Challenges (2025) · cites this paper as [7]

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Authors
Betterle, C., Dal Pra, C., Mantero, F., & Zanchetta, R.
Affiliations
  • University of Padua (IT)
  • Chair of Clinical Immunology and Allergy (C.B., C.D.P., R.Z.), Department of Medical and Surgical Sciences, University of Padova, I-35128, Padova, Italy
ORCID
Venue
Endocrine Reviews
Publisher
The Endocrine Society
Published
2002-06-01
Volume
23
Issue
3
Pages
327-364
ISSN
  • 0163-769X (ISSN-L)
  • 1945-7189
DOI
10.1210/edrv.23.3.0466
OpenAlex
W2125286421
Cited by
758
References
354
Citations by year
2026: 26 · 2025: 16 · 2024: 28 · 2023: 23 · 2022: 24 · 2021: 32 · 2020: 30 · 2019: 39 · 2018: 30 · 2017: 36 · 2016: 28 · 2015: 35 · 2014: 47 · 2013: 36 · 2012: 36
FWCI
4.26
Type
journal article
Language
en
Open access
closed
Locations
  • Endocrine Reviews (publishedVersion)
  • PubMed (publishedVersion)
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 › Neurology › Myasthenia Gravis and Thymoma
Keywords
autoimmune adrenal insufficiency, Addison's disease, autoimmune polyglandular syndrome, autoantibodies, autoantigens, 21-hydroxylase, hypergonadotropic hypogonadism, adrenal imaging
MeSH terms
Adrenal Insufficiency, Autoantibodies, Autoantigens, Autoimmune Diseases, Humans, Prognosis, Polyendocrinopathies, Autoimmune

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