Article: An Update on Addison’s Disease
Experimental and Clinical Endocrinology & Diabetes
2018 Volume 127, Issue 02/03, Page(s) 165–175
Abstract: Addison’s disease – the traditional term for primary adrenal insufficiency (PAI) – is defined ...
| 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. |
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| Keywords | primary adrenal insufficiency ; addison’s disease ; diagnosis ; replacement therapy ; glucocorticoid ; mineralocorticoid ; dehydroepiandrosterone ; adrenal crisis ; clinical practice guideline |
| Language | English |
| Publishing date | 2018-12-18 |
| Publisher | © Georg Thieme Verlag KG |
| Publishing place | Stuttgart ; New York |
| Document type | Article |
| ZDB-ID | 1225416-2 |
| ISSN | 1439-3646 ; 0947-7349 |
| ISSN (online) | 1439-3646 |
| ISSN | 0947-7349 |
| DOI | 10.1055/a-0804-2715 |
| Database | Thieme publisher's database |
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