Addison's disease

This article is about medically-recognized primary adrenal insufficiency. For a term used in alternative medicine, see Adrenal fatigue.

Addison's disease
Other namesAddison disease, primary adrenal insufficiency,[1] primary adrenocortical insufficiency, chronic adrenal insufficiency, chronic adrenocortical insufficiency, primary hypocorticalism, primary hypocortisolism, primary hypoadrenocorticism, primary hypocorticism, primary hypoadrenalism
Darkening of the skin seen on the legs of an otherwise fair-skinned patient
SpecialtyEndocrinology
SymptomsAbdominal pain, weakness, weight loss, darkening of the skin[1]
ComplicationsAdrenal crisis[1]
Usual onsetMiddle-aged females[1]
CausesProblems with the adrenal gland[1]
Diagnostic methodBlood tests, urine tests, medical imaging[1]
TreatmentSynthetic corticosteroid such as hydrocortisone and fludrocortisone[1][2]
Frequency0.9–1.4 per 10,000 people (developed world)[1][3]
DeathsDoubles risk of death
Named afterThomas Addison

Addison's disease, also known as primary adrenal insufficiency,[4] is a rare long-term endocrine disorder characterized by inadequate production of the steroid hormones cortisol and aldosterone by the two outer layers of the cells of the adrenal glands (adrenal cortex), causing adrenal insufficiency.[5][6] Symptoms generally come on slowly and insidiously and may include abdominal pain and gastrointestinal abnormalities, weakness, and weight loss.[1] Darkening of the skin in certain areas may also occur.[1] Under certain circumstances, an adrenal crisis may occur with low blood pressure, vomiting, lower back pain, and loss of consciousness.[1] Mood changes may also occur. Rapid onset of symptoms indicates acute adrenal failure, which is a clinical emergency.[5] An adrenal crisis can be triggered by stress, such as from an injury, surgery, or infection.[1]

Addison's disease arises when the adrenal gland does not produce sufficient amounts of the steroid hormones cortisol and (sometimes) aldosterone.[1] It is an autoimmune disease which affects some genetically predisposed people in whom the body's own immune system has started to target the adrenal glands.[7] In many adult cases it is unclear what has triggered the onset of this disease, though it sometimes follows tuberculosis.[7] Causes can include certain medications, sepsis, and bleeding into both adrenal glands.[1][8] Addison's disease is generally diagnosed by blood tests, urine tests, and medical imaging.[1]

Treatment involves replacing the absent or low hormones.[1] This involves taking a synthetic corticosteroid, such as hydrocortisone or fludrocortisone.[1][2] These medications are typically taken orally.[1] Lifelong, continuous steroid replacement therapy is required, with regular follow-up treatment and monitoring for other health problems which may occur.[9] A high-salt diet may also be useful in some people.[1] If symptoms worsen, an injection of corticosteroid is recommended (people need to carry a dose with them at all times).[1] Often, large amounts of intravenous fluids with the sugar dextrose are also required.[1] With appropriate treatment, the overall outcome is generally favorable,[10] and most people are able to lead a reasonably normal life.[11] Without treatment, an adrenal crisis can result in death.[1]

Addison's disease affects about 9 to 14 per 100,000 people in the developed world.[1][3] It occurs most frequently in middle-aged females.[1] The disease is named after Thomas Addison, a graduate of the University of Edinburgh Medical School, who first described the condition in 1855.[12][13]

  1. ^ a b c d e f g h i j k l m n o p q r s t u v w x "Adrenal Insufficiency and Addison's Disease". National Institute of Diabetes and Digestive and Kidney Diseases. May 2014. Archived from the original on 13 March 2016. Retrieved 13 March 2016.
  2. ^ a b Napier C, Pearce SH (June 2014). "Current and emerging therapies for Addison's disease". Current Opinion in Endocrinology, Diabetes, and Obesity. 21 (3). Lippincott Williams & Wilkins Ltd.: 147–153. doi:10.1097/med.0000000000000067. PMID 24755997. S2CID 13732181. Archived from the original on 2019-10-31. Retrieved 2019-10-31.
  3. ^ a b Brandão Neto RA, de Carvalho JF (2014). "Diagnosis and classification of Addison's disease (autoimmune adrenalitis)". Autoimmunity Reviews. 13 (4–5): 408–411. doi:10.1016/j.autrev.2014.01.025. PMID 24424183.
  4. ^ Oelkers W (2000). "Clinical diagnosis of hyper- and hypocortisolism". Noise & Health. 2 (7): 39–48. PMID 12689470. Archived from the original on 2022-08-10. Retrieved 2021-03-03.
  5. ^ a b "Addison's Disease". NORD (National Organization for Rare Disorders). Archived from the original on 2022-06-15. Retrieved 2020-12-01.
  6. ^ Kuo B, Koransky A, Vaz Wicks CL (2023-03-01). "Adrenal Crisis as An Adverse Reaction to Zoledronic Acid in a Patient With Primary Adrenal Insufficiency: A Case Report and Literature Review". AACE Clinical Case Reports. 9 (2): 32–34. doi:10.1016/j.aace.2022.12.003. PMC 10086596. PMID 37056413.
  7. ^ a b Hellesen A, Bratland E (January 2019). "The potential role for infections in the pathogenesis of autoimmune Addison's disease". Clinical and Experimental Immunology. 195 (1): 52–63. doi:10.1111/cei.13207. PMC 6300649. PMID 30144040.
  8. ^ Adam A (2014). Grainger & Allison's Diagnostic Radiology (6 ed.). Elsevier Health Sciences. p. 1031. ISBN 9780702061288. Archived from the original on 14 March 2016.
  9. ^ Napier C, Pearce SH (December 2012). "Autoimmune Addison's disease". Presse Médicale. 41 (12 P 2). Elsevier: e626–e635. doi:10.1016/j.lpm.2012.09.010. PMID 23177474.
  10. ^ Rajagopalan SR, Longmore M, Wilkinson IB (2006). Mini Oxford handbook of clinical medicine (6 ed.). Oxford: Oxford University Press. p. 312. ISBN 9780198570714. Archived from the original on 14 March 2016.
  11. ^ Cite error: The named reference NHS was invoked but never defined (see the help page).
  12. ^ Ten S, New M, Maclaren N (July 2001). "Clinical review 130: Addison's disease 2001". The Journal of Clinical Endocrinology and Metabolism. 86 (7): 2909–2922. doi:10.1210/jcem.86.7.7636. PMID 11443143.
  13. ^ Rose NR, Mackay IR (2014). The autoimmune diseases (5 ed.). San Diego, CA: Elsevier Science. p. 605. ISBN 9780123849304. Archived from the original on 14 March 2016.