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.
Experimental and clinical endocrinology & diabetes : official journal, German Society of Endocrinology [and] German Diabetes Association. 2018 Dec 18 [Epub ahead of print]
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
Medicover, Bochum, Germany., Medicover, Berlin-Mitte, Germany., Medicover, Ulm and Neu-Ulm, Germany., Department of Medicine III, University Hospital Carl Gustav Carus, Dresden, Germany., University of Calgary, Calgary, AB,Canada., Department of Medicine I, Würzburg University Hospital, Würzburg, Germany., Klinik für Endokrinologie, Diabetologie und Klinische Ernährung, University Hospital, Zürich, Switzerland., Division of Diabetes & Nutritional Sciences, Faculty of Life Sciences & Medicine, King's College London, London, United Kingdom., Lee Kong Chian School of Medicine, NTU Nanyang Technological University, Singapore, Singapore., Endocrine and Metabolic Unit, Royal Adelaide Hospital, University of Adelaide, Adelaide SA, Australia.