An Update on Addison’s Disease

Andreas Barthel; Georg Benker; Kai Berens; Sven Diederich; Burkhard Manfras; Matthias Gruber; Waldemar Kanczkowski; Greg Kline; Virginia Kamvissi-Lorenz; Stefanie Hahner; Felix Beuschlein; Ana Brennand; Bernhard O. Boehm; David J. Torpy; Stefan R. Bornstein

doi:10.1055/a-0804-2715

Experimental and Clinical Endocrinology & Diabetes, Table of Contents

Exp Clin Endocrinol Diabetes 2019; 127(02/03): 165-175
DOI: 10.1055/a-0804-2715

Review

An Update on Addison’s Disease

Authors

Andreas Barthel

¹Medicover, Bochum, Germany

²Department of Medicine III, University Hospital Carl Gustav Carus, Dresden, Germany
Georg Benker

¹Medicover, Bochum, Germany
Kai Berens

¹Medicover, Bochum, Germany
Sven Diederich

³Medicover, Berlin-Mitte, Germany
Burkhard Manfras

⁴Medicover, Ulm and Neu-Ulm, Germany
Matthias Gruber

²Department of Medicine III, University Hospital Carl Gustav Carus, Dresden, Germany
Waldemar Kanczkowski

²Department of Medicine III, University Hospital Carl Gustav Carus, Dresden, Germany
Greg Kline

⁵University of Calgary, Calgary, AB,Canada
Virginia Kamvissi-Lorenz

²Department of Medicine III, University Hospital Carl Gustav Carus, Dresden, Germany

⁸Division of Diabetes & Nutritional Sciences, Faculty of Life Sciences & Medicine, King’s College London, London, United Kingdom
Stefanie Hahner

⁶Department of Medicine I, Würzburg University Hospital, Würzburg, Germany
Felix Beuschlein

⁷Klinik für Endokrinologie, Diabetologie und Klinische Ernährung, University Hospital, Zürich, Switzerland
Ana Brennand

⁸Division of Diabetes & Nutritional Sciences, Faculty of Life Sciences & Medicine, King’s College London, London, United Kingdom
Bernhard O. Boehm

⁹Lee Kong Chian School of Medicine, NTU Nanyang Technological University, Singapore, Singapore
David J. Torpy

¹⁰Endocrine and Metabolic Unit, Royal Adelaide Hospital, University of Adelaide, Adelaide SA, Australia
Stefan R. Bornstein

²Department of Medicine III, University Hospital Carl Gustav Carus, Dresden, Germany

⁷Klinik für Endokrinologie, Diabetologie und Klinische Ernährung, University Hospital, Zürich, Switzerland

⁸Division of Diabetes & Nutritional Sciences, Faculty of Life Sciences & Medicine, King’s College London, London, United Kingdom

⁹Lee Kong Chian School of Medicine, NTU Nanyang Technological University, Singapore, Singapore

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

Full Text

References

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