What is the primary treatment for adrenal insufficiency?

From the Guidelines

The primary treatment for adrenal insufficiency is hormone replacement therapy with glucocorticoids and, if needed, mineralocorticoids, with hydrocortisone being the most commonly prescribed glucocorticoid at a total daily dose of 15-25 mg divided into 2-3 doses. This treatment approach is based on the most recent and highest quality study available, which emphasizes the importance of mimicking the natural cortisol rhythm to manage adrenal insufficiency effectively 1.

Key Considerations for Treatment

• Hydrocortisone is typically given in a higher dose in the morning and a lower dose in the evening to mimic the natural cortisol rhythm.
• For mineralocorticoid replacement, fludrocortisone (Florinef) is prescribed at a dose of 0.05-0.2 mg once daily for patients with primary adrenal insufficiency who have aldosterone deficiency.
• Patients must carry emergency medication (injectable hydrocortisone) and wear medical alert identification, as stress situations require dose adjustments—typically doubling or tripling the glucocorticoid dose during minor illness with fever, and using injectable forms during severe illness, trauma, or vomiting.
• Regular monitoring of symptoms, blood pressure, electrolytes, and plasma renin activity helps optimize treatment.

Importance of Education and Monitoring

• All patients need education on stress dosing for sick days, use of emergency steroid injectables, when to seek medical attention for impending adrenal crisis, and a medical alert bracelet for adrenal insufficiency to trigger stress dose corticosteroids by emergency medical personnel.
• Early endocrinology consultation is appropriate for planning before surgery or high-stress treatments.
• Laboratory confirmation of adrenal insufficiency should be approached with caution in patients given high-dose corticosteroids for other conditions, and consultation with endocrinology is recommended for recovery and weaning protocols using hydrocortisone in patients with symptoms of adrenal insufficiency after weaning off corticosteroids 1.

Treatment Adjustments and Considerations

• Treatment adjustments may be necessary based on the severity of symptoms, with more severe cases potentially requiring inpatient management and higher doses of hydrocortisone.
• The relationship between ACTH and cortisol levels can help distinguish between primary and secondary adrenal insufficiency, guiding the management approach 1.
• Using hydrocortisone allows for the recreation of the diurnal rhythm of cortisol, which is crucial for managing adrenal insufficiency effectively.

From the FDA Drug Label

Endocrine Disorders Primary or secondary adrenocortical insufficiency (hydrocortisone or cortisone is the first choice; synthetic analogs may be used in conjunction with mineralocorticoids where applicable; in infancy, mineralocorticoid supplementation is of particular importance) The primary treatment for adrenal insufficiency is hydrocortisone or cortisone as the first choice, with synthetic analogs and mineralocorticoids used in conjunction where applicable 2.

• Key points:
  • Hydrocortisone or cortisone is the first choice for treatment
  • Synthetic analogs may be used in conjunction with mineralocorticoids
  • Mineralocorticoid supplementation is particularly important in infancy

From the Research

Adrenal Insufficiency Treatment

The primary treatment for adrenal insufficiency involves supplemental glucocorticoids, such as hydrocortisone (15-25 mg daily) or prednisone (3-5 mg daily) 3.

• For patients with primary adrenal insufficiency, mineralocorticoids (e.g., fludrocortisone, 0.05-0.3 mg daily) should be added to the treatment regimen 3.
• The treatment goal is to replicate the natural circadian rhythm of cortisol release and provide individualized cortisol exposure 4.
• Patients with adrenal insufficiency should be instructed on how to increase glucocorticoids during acute illness and prescribed injectable glucocorticoids (e.g., hydrocortisone, 100 mg intramuscular injection) to prevent or treat adrenal crisis 3, 5.

Special Considerations

• In stressful situations, such as surgery or sepsis, patients with adrenal insufficiency require increased hydrocortisone cover to avoid adrenal crisis 5.
• Continuous intravenous hydrocortisone infusion may be the most effective mode of administration in these situations, as it can maintain cortisol concentrations in the required range 5.
• Women with adrenal insufficiency may benefit from additional substitution of dehydroepiandrosterone (DHEA) to improve well-being and sexual function 6.

Long-term Management

• The optimization of hormone replacement therapy is crucial to improve health-related quality of life and reduce mortality and morbidity in patients with primary adrenal insufficiency 7.
• Regular dose adaptation and monitoring are necessary to prevent over- or under-treatment, which can result in Cushing-like symptoms or adrenal crisis, respectively 4.