Addison's disease

Presentation may be acute or insidious, with substantial fatigue and weakness associated with mucocutaneous hyperpigmentation, hypotension and/or postural hypotension, and salt craving.

Adrenocorticotrophic hormone stimulation test is performed to confirm or exclude the diagnosis of Addison's disease.

All patients receive mineralocorticoid and glucocorticoid replacement for life, and are instructed to increase the dose of glucocorticoid during surgery and during any stressful or infectious conditions.

Treatment complications arise from over-replacement of mineralocorticoid and/or glucocorticoid.

Addison's disease, or primary adrenal insufficiency, is a disorder that affects the adrenal glands, causing decreased production of adrenocortical hormones (cortisol, aldosterone, and dehydroepiandrosterone).[1]Bornstein SR, Allolio B, Arlt W, et al. Diagnosis and treatment of primary adrenal insufficiency: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2016 Feb;101(2):364-89. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4880116 http://www.ncbi.nlm.nih.gov/pubmed/26760044?tool=bestpractice.com This may be caused by a destructive process directly affecting the adrenal glands or by a condition that interferes with hormone synthesis. Approximately 90% of the adrenal cortex needs to be destroyed to produce adrenal insufficiency. Addison's disease may be either acute (adrenal crisis) or insidious. If untreated, it is a potentially life-threatening condition.

In contrast to Addison's disease, secondary adrenal insufficiency occurs in patients with pituitary or hypothalamic involvement. This results in decreased adrenocorticotrophic hormone secretion, which ultimately results in adrenal failure. Adrenal deficiency in children is not specifically covered in this topic, but should result in referral to a paediatric endocrinology specialist.