|Other names||Addisonian crisis, acute adrenal insufficiency|
|Forty-nine year-old with an adrenal crisis. Appearance shows lack of facial hair, dehydration, loss of the outer eyebrow (panel A), pale skin, muscular and weight loss, and loss of body hair (panel B).|
|Specialty||Emergency medicine, endocrinology|
|Symptoms||Confusion, abdominal pain, nausea, vomiting, fever, low blood pressure|
|Complications||Seizures, irregular heart rate, organ failure|
|Causes||Triggering event in adrenal insufficiency|
|Diagnostic method||Supported by: High blood potassium, low blood sodium, low cortisol level|
Confirmed by: ACTH stimulation test
|Differential diagnosis||Shock, heart attack, myxedema coma, gastroenteritis|
|Prevention||Doubling usual dose of steroids when unwell|
|Treatment||Hydrocortisone, intravenous fluids, vasopressors, dextrose, antibiotics|
|Prognosis||Risk of death up to 25%|
|Frequency||3 in 100,000 per year|
Adrenal crisis is a complication of adrenal insufficiency that may occur following a triggering event. Symptoms are of sudden onset and may include confusion, abdominal pain, nausea, vomiting, fever, and low blood pressure. Complications may include seizures, irregular heart rate, and organ failure.
Adrenal insufficiency may occur due to Addison's disease, Waterhouse–Friderichsen syndrome, chronic glucocorticoid use, or pituitary apoplexy. However, in more than half of crisis people do not realize that they have adrenal insufficiency. Triggers may include stopping corticosteroids after prolonged use, infection, injury, thyrotoxicosis, strenuous exercise, alcohol, and emotional stress. The underlying mechanism involves not enough of the hormone cortisol. Diagnosis is supported by a high blood potassium, low blood sodium, and low cortisol level. Confirmation is by an ACTH stimulation test.
Prevention is by doubling a person's usual dose of steroids when unwell. Treatment is generally by giving an injection of hydrocortisone, though methylprednisolone or dexamethasone may also be used. Intravenous fluids, vasopressors, dextrose, and antibiotics may also be needed.
Primary adrenal insufficiency affects about 5 per 100,000 people while secondary adrenal insufficiency affects about 22 per 100,000 people. In those with adrenal insufficiency adrenal crisis occurs in about 5% to 10% a year. Older people are more commonly affected. In those with adrenal insufficiency 1 in 200 die per year from this complication. During an adrenal crisis the risk of death is as high as 25%.
Signs and symptoms
Characteristic symptoms are:
- Sudden onset pain in the legs, lower back or abdomen
- Confusion, psychosis, slurred speech
- Severe lethargy
- Low blood pressure
- 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)
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
Laboratory changes may include:
- 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)
- Hypothyroid (low T4 level)
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. Medication should be given within two hours of trauma and consequently it is advisable to carry injectable hydrocortisone in remote areas. This is known as stress dose steroids.
- 1L of 0.9% saline over 30-60 min with 100mg of i.v. Bolus hydrocortisone.
- Continuous infusion of saline within 24hours with 100mg I.m. hydrocortisone 6-hourly.
- To be shifted to oral medication based on the person’s state. Hydrocortisone 20mg 8-hourly reduced to 20-30mg in divided doses over few days.
- Fludrocortisone is given later.
Adrenal insufficiency affects about 1 in 20,000 people. Women are affected about twice as often as men. In those with adrenal insufficiency adrenal crisis occurs in about 5% to 10% a year. Older people are more commonly affected.
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- Hydrocortisone The Pituitary Foundation, UK
- Adrenal crisis on Patient.info