What is the treatment for adrenal insufficiency?

Treatment for Adrenal Insufficiency

The standard treatment for adrenal insufficiency consists of glucocorticoid replacement with hydrocortisone 15-25 mg daily in divided doses, plus mineralocorticoid replacement with fludrocortisone 50-200 μg daily for primary adrenal insufficiency. 1

Glucocorticoid Replacement Therapy

• Hydrocortisone is the preferred glucocorticoid replacement, typically given in a total daily dose of 15-25 mg divided into 2-3 doses 1
• The most common dosing schedule is a three-dose regimen: 10 mg on waking, 5 mg at noon, and 2.5-5 mg in late afternoon to mimic the natural cortisol rhythm 1
• Alternative glucocorticoids include cortisone acetate (25-37.5 mg daily in divided doses) 1
• Prednisolone (4-5 mg daily) may be considered in cases of compliance problems, marked energy fluctuations, or when hydrocortisone is not tolerated 1
• Dexamethasone is generally not recommended for routine replacement therapy as it lacks mineralocorticoid activity and has a long half-life that can disrupt the normal cortisol rhythm 2

Mineralocorticoid Replacement Therapy

• Fludrocortisone is indicated for all patients with primary adrenal insufficiency at a dose of 50-200 μg (0.05-0.2 mg) once daily, usually taken upon awakening 1, 3
• Higher doses (up to 500 μg daily) may be needed in children, younger adults, or during the last trimester of pregnancy 1, 4
• Dose adequacy is assessed by monitoring:
  • Blood pressure (both supine and standing positions) 4
  • Serum electrolytes 1, 4
  • Clinical symptoms such as salt cravings, lightheadedness, and peripheral edema 4

Adrenal Crisis Management

• Adrenal crisis requires immediate treatment with:
  • Intravenous or intramuscular hydrocortisone 100 mg, followed by 100 mg every 6-8 hours until recovery 1
  • Isotonic (0.9%) sodium chloride solution at an initial rate of 1 L/hour until hemodynamic improvement 1
  • Identification and treatment of the underlying precipitant (e.g., infection) 1

Dose Adjustments for Special Situations

• During surgery or major medical procedures:
  • Intravenous or intramuscular hydrocortisone and increased oral doses are required 1
• Stress dose adjustments:
  • During acute illness or physical stress, glucocorticoid doses should be increased 2-3 fold 5, 6
• Medication interactions requiring dose adjustments:
  • Drugs that may increase hydrocortisone requirements: anti-epileptics, antituberculosis medications, etomidate, and topiramate 1
  • Drugs that may decrease hydrocortisone requirements: grapefruit juice and licorice 1
  • NSAIDs should be avoided with fludrocortisone 1, 4
  • Drospirenone-containing contraceptives may require higher fludrocortisone doses 4

Patient Education and Monitoring

• All patients should:
  • Wear medical alert identification jewelry 1
  • Carry a steroid alert card 1
  • Have supplies for self-injection of parenteral hydrocortisone 1
  • Receive education on managing daily medications and minor illnesses 1
• Annual follow-up should include:
  • Serum electrolyte monitoring 1, 4
  • Screening for development of other autoimmune disorders, particularly hypothyroidism 1
  • Bone mineral density monitoring every 3-5 years 1

Common Pitfalls and Caveats

• Under-replacement with mineralocorticoids is common and sometimes compensated for by over-replacement with glucocorticoids 1
• Essential hypertension in a patient with primary adrenal insufficiency should be treated by adding a vasodilator and reducing (not stopping) the fludrocortisone dose 1, 4
• Patients should eat sodium salt and salty foods without restriction and avoid potassium-containing salts 1, 4
• Current conventional hydrocortisone replacement regimens often result in suboptimal treatment with many patients having cortisol levels outside the physiological range 7
• The short half-life of hydrocortisone (approximately 1.5 hours) necessitates multiple daily dosing to avoid high peaks and low troughs 8
• DHEA supplementation may benefit some women with adrenal insufficiency regarding well-being and sexual function 6