Adrenal Crisis

Adrenal crisis is a potentially life-threatening medical condition 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
• 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.
• 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 (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.

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.

Treatment

Acute adrenal insufficiency is a medical emergency and needs to be treated with injectable hydrocortisone and fluid support.

1. 1L of 0.9% saline over 30-60 min with 100mg of i.v. Bolus hydrocortisone.
2. Continuous infusion of saline within 24hours with 100mg I.m. hydrocortisone 6-hourly.
3. Glucose
4. To be shifted to oral medication based on the patient’s state. Hydrocortisone 20mg 8-hourly reduced to 20-30mg in divided doses over few days.
5. Fludrocortisone is given later.

Epidemiology

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

See also

• Stress dose