What are the causes and management of adrenal crisis (adrenal insufficiency)?

From the Guidelines

Adrenal crisis is a life-threatening medical emergency requiring immediate treatment with hydrocortisone 100mg IV bolus, followed by 200mg over 24 hours as a continuous infusion, as recommended by the most recent guidelines from the Association of Anaesthetists, the Royal College of Physicians, and the Society for Endocrinology UK 1.

Key Recommendations

• Administer hydrocortisone 100mg IV bolus at the start of surgery, followed by a continuous infusion of 200mg over 24 hours 1.
• Double the regular oral replacement dose of hydrocortisone for 48 hours, and for up to a week following major surgery, before resuming the maintenance dose 1.
• Patients with adrenal insufficiency should always carry an emergency injection kit containing hydrocortisone 100mg and be trained in self-administration 1.
• They should wear medical alert identification and double or triple their usual steroid dose during illness, surgery, or significant stress 1.

Prevention and Education

• Patients with adrenal insufficiency are at risk of adrenal crisis, especially during periods of physiological stress, such as illness, surgery, or trauma 1.
• Early recognition and treatment of adrenal crisis are crucial to prevent mortality and morbidity 1.
• Patients should receive comprehensive education on prevention strategies, including sick day rules, and regular follow-up with an endocrinologist 1.

Treatment and Management

• Treatment of adrenal crisis should include immediate administration of hydrocortisone and fluid replacement with isotonic saline or 5% dextrose in isotonic saline 1.
• The dose of hydrocortisone may need to be adjusted based on the patient's response to treatment and the presence of other medical conditions, such as diabetes or hypertension 1.

From the FDA Drug Label

To avoid drug-induced adrenal insufficiency, supportive dosage may be required in times of stress (such as trauma, surgery, or severe illness) both during treatment with fludrocortisone acetate and for a year afterwards Drug-induced secondary adrenocortical insufficiency may result from too rapid withdrawal of corticosteroids and may be minimized by gradual reduction of dosage. Increased dosage of rapidly acting corticosteroids is indicated in patients on corticosteroid therapy subjected to any unusual stress before, during, and after the stressful situation Drug induced secondary adrenocortical insufficiency may be minimized by gradual reduction of dosage. This type of relative insufficiency may persist for months after discontinuation of therapy; therefore, in any situation of stress occurring during that period, hormone therapy should be reinstituted

Adrenal crises can be triggered by the withdrawal of corticosteroids, and to minimize this risk, a gradual reduction in dosage is recommended. In situations of stress, an increase in dosage of rapidly acting corticosteroids may be necessary. Patients on corticosteroid therapy should be advised to carry medical identification indicating their dependence on steroid medication and to have an adequate supply of medication for use in emergencies 2, 3, 4.

• Key points:
  • Gradual reduction in dosage to minimize adrenal insufficiency
  • Increased dosage in times of stress
  • Medical identification and emergency medication supply
• Main idea: Adrenal crises can occur with corticosteroid withdrawal or stress, and preventive measures should be taken.

From the Research

Definition and Incidence of Adrenal Crisis

• Adrenal crisis is a life-threatening emergency that contributes to the excess mortality of patients with adrenal insufficiency 5, 6, 7.
• The incidence of adrenal crisis is estimated to be 5-10 per 100 patient years in patients with adrenal insufficiency, with a mortality rate of 0.5 per 100 patient years 5.
• The incidence of adrenal crisis is also estimated to be 8 per 100 patient years in patients with adrenal insufficiency 7.

Causes and Precipitating Factors of Adrenal Crisis

• Infections are the major precipitating cause of adrenal crisis 5, 6, 7.
• Other causes include physical stress such as surgical procedures or trauma, forgetting or discontinuing glucocorticoid therapy, pronounced physical activity, and psychological stress 6, 7.
• Emotional distress, surgery, cessation or reduction in glucocorticoid doses, pituitary infarction, or surgical cure of endogenous Cushing's syndrome can also precipitate adrenal crisis 6.

Symptoms and Diagnosis of Adrenal Crisis

• Patients with adrenal crisis typically present with profoundly impaired well-being, hypotension, nausea and vomiting, and fever 5.
• The diagnosis of adrenal crisis can be challenging, especially in patients not known to have adrenal insufficiency, and may be delayed due to non-specific symptoms and signs 6, 7.

Treatment and Management of Adrenal Crisis

• The treatment of adrenal crisis involves prompt recognition and administration of parenteral hydrocortisone, rehydration, and management of electrolyte abnormalities 5, 6, 7.
• The recommended treatment is an initial bolus of 100 mg hydrocortisone followed by 200 mg over 24 hours as a continuous infusion 5, 6.
• Continuous intravenous hydrocortisone infusion is the most appropriate mode of administration for maintaining cortisol concentrations in the required range during major stress 8.

Prevention of Adrenal Crisis

• Prevention of adrenal crisis requires appropriate hydrocortisone dose adjustments to stressful medical procedures and other stressful events 5.
• Patient education is a key factor in preventing adrenal crisis, and patients should be educated on stress dosing and parenteral glucocorticoid administration 5, 6, 7.
• Patients should carry a steroid dependency alert card and wear a medical alert bracelet or similar identification 7.