Adrenal crisis (also known as Addisonian crisis and acute adrenal insufficiency) is a medical emergency and potentially life-threatening situation requiring immediate emergency treatment. It is a constellation of symptoms that indicate severe adrenal insufficiency caused by insufficient levels of the hormone cortisol. This may be the result of either previously undiagnosed or untreated Addison's disease, a disease process suddenly affecting adrenal function (such as bleeding from the adrenal glands in Waterhouse-Friderichsen syndrome), suddenly stopping intake of glucocorticoids or an intercurrent problem (e.g. infection, trauma, in fact any form of physical or mental stress) in someone known to have Addison's disease, congenital adrenal hyperplasia (CAH), or other form of primary adrenal insufficiency.
Signs and symptoms
Characteristic symptoms are:
- Sudden penetrating pain in the legs, lower back or abdomen
- Confusion, psychosis, slurred speech
- Severe lethargy
- 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.
- Hypoglycemia (reduced level of blood glucose)
- Hyponatremia (low sodium level in the blood)
- Hypotension (low blood pressure)
- Hypothyroid (low T4 level)
- Severe vomiting and diarrhea, resulting in dehydration
- Syncope (loss of consciousness and ability to stand)
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.
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
Adrenal crisis is triggered by physiological stress (such as trauma). 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.
Hahner et al. investigated the frequency, causes and risk factors for adrenal crisis in patients with chronic adrenal insufficiency. Annane et al.'s landmark 2002 study found a very high rate of relative adrenal insufficiency among the enrolled patients with septic shock.
- "Acute adrenal crisis (Addisonian crisis)". Endocrine Surgery Encyclopedia. UCLA Health System. Retrieved 14 August 2013.
- "Addison's Disease". National Endocrine and Metabolic Diseases Information Service. Retrieved 14 August 2013.
- "Etiology of hypercalcemia in a patient with Addison's disease". Calcified Tissue International. 34: 523–526. doi:10.1007/BF02411297.
- Hydrocortisone The Pituitary Foundation, UK
- Hahner, S.; Loeffler, M.; Bleicken, B.; Drechsler, C.; Milovanovic, D.; Fassnacht, M.; Ventz, M.; Quinkler, M.; Allolio, B. (2 December 2009). "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.
- Annane, D. (20 August 2002). "Effect of Treatment With Low Doses of Hydrocortisone and Fludrocortisone on Mortality in Patients With Septic Shock". JAMA: The Journal of the American Medical Association. 288 (7): 862–871. doi:10.1001/jama.288.7.862. PMID 12186604.