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

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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

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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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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: Betterle, Corrado
Mantero, Franco
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 › Clinical 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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Autoimmune Adrenal Insufficiency and Autoimmune Polyendocrine Syndromes: Autoantibodies, Autoantigens, and Their Applicability in Diagnosis and Disease Prediction

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