Adrenal crisis

Adrenal crisis is a potentially life-threatening medical condition requiring immediate emergency treatment.[2] It is a constellation of symptoms (caused by insufficient levels of the hormone cortisol) that indicate severe adrenal insufficiency.[3] This may be the result of either previously undiagnosed or untreated Addison's disease, a disease process that suddenly affects adrenal function (such as bleeding from the adrenal glands, as in the case of Waterhouse–Friderichsen syndrome), suddenly stopping intake of glucocorticoids or an intercurrent problem (e.g. infection, trauma, or other forms of physical or mental stress) in someone known to have Addison's disease, congenital adrenal hyperplasia (CAH), or another form of primary adrenal insufficiency.

Adrenal crisis
Other namesAddisonian crisis and acute adrenal insufficiency
A chart measuring a change in plasma cortisol cycle (mcg/dl) over 24 hours
SpecialtyEmergency medicine, Endocrinology
SymptomsFever, lethargy, hyperkalemia, hyponatremia, hypotension, diarrhea, vomiting, convulsions[1]
CausesAddison's disease, Congenital adrenal hyperplasia, Waterhouse–Friderichsen syndrome, hypopituitarism

Signs and symptoms

Characteristic symptoms are:[4]

  • Sudden penetrating pain in the legs, lower back or abdomen
  • Confusion, psychosis, slurred speech
  • Severe lethargy and generally "not feeling well"[5]
  • Convulsions
  • Fever
  • Hyperkalemia (elevated potassium level in the blood)
  • Hypercalcemia (elevated calcium level in the blood): the cause of hypercalcemia is a combination of increased calcium input into the extracellular space and reduced calcium removal by the kidney, this last caused by decreased glomerular filtration and increased tubular calcium reabsorption. Both renal factors are secondary to volume depletion and, in fact, improve rapidly during rehydration with saline infusion.[6]
  • Hypoglycemia (reduced level of blood glucose)
  • Hyponatremia (low sodium level in the blood)
  • Hypotension (low blood pressure)
  • Hypothyroidism (low T4 level)
  • Severe vomiting and diarrhea, resulting in dehydration
  • Syncope (transient loss of consciousness) and/or orthostatic hypotension (drop in blood pressure on standing, leading to loss of balance)

Causes

Adrenal crisis is caused by a deficiency of cortisol resulting from Addison's disease, congenital adrenal hyperplasia (CAH), corticosteroid biosynthetic enzyme defects or pituitary disorders (such as Sheehan's syndrome, pituitary adenoma, hypopituitarism (inactive or underactive pituitary) causing failure to activate the adrenal glands. May also be a side effect of Zytiga (Abiraterone) for prostate cancer.

Diagnosis

Various investigations aid the diagnosis:

  • ACTH (cosyntropin) stimulation test
  • Cortisol level (to assess the level of glucocorticoids)
  • Fasting blood sugar
  • Serum potassium (to assess the level of mineralocorticoids)
  • Serum sodium

Prevention

Adrenal crisis is triggered by physiological stress (such as trauma) or severe psychological stress. Activities that have an elevated risk of trauma are best avoided. Treatment must be given within two hours of trauma and consequently it is advisable to carry injectable hydrocortisone in remote areas.[7]

Treatment

Acute adrenal insufficiency is a medical emergency and needs to be treated with injectable hydrocortisone and fluid support.[3][8][9][10]

  1. 1L of 0.9% saline over 30-60 min with 100 mg of i.v. hydrocortisone (bolus)
  2. Continuous infusion of normal saline plus 100 mg i.m. hydrocortisone every 6 hours.
  3. Glucose
  4. Shift to p.o. medication based on the patient's state: hydrocortisone 20 mg every 8 hours; reduce by 20–30 mg (in divided doses) over the following few days and then discontinue (if appropriate).
  5. Begin fludrocortisone.

Epidemiology

Hahner et al. investigated the frequency, causes and risk factors for adrenal crisis in patients with chronic adrenal insufficiency.[11]

See also

References
  1. "Acute adrenal crisis". MedlinePlus Medical Encyclopedia. U.S. National Library of Medicine.
  2. 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.
  3. "Acute adrenal crisis (Addisonian crisis)". Endocrine Surgery Encyclopedia. UCLA Health System. Retrieved 14 August 2013.
  4. "Addison's Disease". National Endocrine and Metabolic Diseases Information Service. Archived from the original on 28 October 2007. Retrieved 14 August 2013.
  5. Dineen R, Thompson CJ, Sherlock M (2019-06-13). "Adrenal crisis: prevention and management in adult patients". Therapeutic Advances in Endocrinology and Metabolism. 10: 2042018819848218. doi:10.1177/2042018819848218. PMC 6566489. PMID 31223468.
  6. Muls E, Bouillon R, Boelaert J, Lamberigts G, Van Imschoot S, Daneels R, De Moor P (1982). "Etiology of hypercalcemia in a patient with Addison's disease". Calcified Tissue International. 34 (6): 523–526. doi:10.1007/BF02411297. PMID 6819071. S2CID 11384341.
  7. Hydrocortisone The Pituitary Foundation, UK
  8. 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.
  9. Dineen R, Thompson CJ, Sherlock M (2019-06-13). "Adrenal crisis: prevention and management in adult patients". Therapeutic Advances in Endocrinology and Metabolism. 10: 2042018819848218. doi:10.1177/2042018819848218. PMC 6566489. PMID 31223468.
  10. Allolio B (March 2015). "Extensive expertise in endocrinology. Adrenal crisis". European Journal of Endocrinology. 172 (3): R115–R124. doi:10.1530/EJE-14-0824. PMID 25288693.
  11. Hahner S, Loeffler M, Bleicken B, Drechsler C, Milovanovic D, Fassnacht M, et al. (March 2010). "Epidemiology of adrenal crisis in chronic adrenal insufficiency: the need for new prevention strategies". European Journal of Endocrinology. 162 (3): 597–602. doi:10.1530/EJE-09-0884. PMID 19955259.
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