An Update on Addison's Disease.

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.

• Authors: 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
• Journal: Experimental and clinical endocrinology & diabetes : official journal, German Society of Endocrinology [and] German Diabetes Association (Exp Clin Endocrinol Diabetes) Vol. 127 Issue 2-03 Pg. 165-175 (Feb 2019) ISSN: 1439-3646 [Electronic] Germany
• PMID: 30562824 (Publication Type: Journal Article, Review)
• Copyright: © Georg Thieme Verlag KG Stuttgart · New York.

Topics

• Addison Disease (diagnosis, drug therapy, epidemiology, etiology)
• Humans