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Exp Clin Endocrinol Diabetes 2019; 127(02/03): 165-175
DOI: 10.1055/a-0804-2715
Review
© Georg Thieme Verlag KG Stuttgart · New York

An Update on Addison’s Disease

Andreas Barthel
1   Medicover, Bochum, Germany
2   Department of Medicine III, University Hospital Carl Gustav Carus, Dresden, Germany
,
Georg Benker
1   Medicover, Bochum, Germany
,
Kai Berens
1   Medicover, Bochum, Germany
,
Sven Diederich
3   Medicover, Berlin-Mitte, Germany
,
Burkhard Manfras
4   Medicover, Ulm and Neu-Ulm, Germany
,
Matthias Gruber
2   Department of Medicine III, University Hospital Carl Gustav Carus, Dresden, Germany
,
Waldemar Kanczkowski
2   Department of Medicine III, University Hospital Carl Gustav Carus, Dresden, Germany
,
Greg Kline
5   University of Calgary, Calgary, AB,Canada
,
Virginia Kamvissi-Lorenz
2   Department of Medicine III, University Hospital Carl Gustav Carus, Dresden, Germany
8   Division of Diabetes & Nutritional Sciences, Faculty of Life Sciences & Medicine, King’s College London, London, United Kingdom
,
Stefanie Hahner
6   Department of Medicine I, Würzburg University Hospital, Würzburg, Germany
,
Felix Beuschlein
7   Klinik für Endokrinologie, Diabetologie und Klinische Ernährung, University Hospital, Zürich, Switzerland
,
Ana Brennand
8   Division of Diabetes & Nutritional Sciences, Faculty of Life Sciences & Medicine, King’s College London, London, United Kingdom
,
Bernhard O. Boehm
9   Lee Kong Chian School of Medicine, NTU Nanyang Technological University, Singapore, Singapore
,
David J. Torpy
10   Endocrine and Metabolic Unit, Royal Adelaide Hospital, University of Adelaide, Adelaide SA, Australia
,
Stefan R. Bornstein
2   Department of Medicine III, University Hospital Carl Gustav Carus, Dresden, Germany
7   Klinik für Endokrinologie, Diabetologie und Klinische Ernährung, University Hospital, Zürich, Switzerland
8   Division of Diabetes & Nutritional Sciences, Faculty of Life Sciences & Medicine, King’s College London, London, United Kingdom
9   Lee Kong Chian School of Medicine, NTU Nanyang Technological University, Singapore, Singapore
› Author Affiliations
Further Information

Publication History

received   05 July 2018
revised   21 November 2018

accepted   21 November 2018

Publication Date:
18 December 2018 (online)

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Abstract

Addison’s disease – the traditional term for primary adrenal insufficiency (PAI) – is defined as the clinical manifestation of chronic glucocorticoid- and/or mineralocorticoid deficiency due to failure of the adrenal cortex which may result in an adrenal crisis with potentially life-threatening consequences. Even though efficient and safe pharmaceutical preparations for the substitution of endogenous gluco- and mineralocorticoids are established in therapy, the mortality in patients with PAI is still increased and the health-related quality of life (HRQoL) is often reduced.

PAI is a rare disease but recent data report an increasing prevalence. In addition to the common “classical” causes of PAI like autoimmune, infectious, neoplastic and genetic disorders, other iatrogenic conditions – mostly pharmacological side effects (e. g., adrenal haemorrhage associated with anticoagulants, drugs affecting glucocorticoid synthesis, action or metabolism and some of the novel anti-cancer checkpoint inhibitors) are contributing factors to this phenomenon.

Due to the rarity of the disease and often non-specific symptoms at least in the early stages, PAI is frequently not considered resulting in a delayed diagnosis. Successful therapy is mainly based on adequate patient education as a cornerstone in the prevention and management of adrenal crisis. A focus of current research is in the development of pharmacokinetically optimized glucocorticoid preparations as well as regenerative therapies.

Key words

primary adrenal insufficiency - addison’s disease - diagnosis - replacement therapy - glucocorticoid - mineralocorticoid - dehydroepiandrosterone - adrenal crisis - clinical practice guideline
 

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