What is the treatment for Addison's disease?

Treatment for Addison's Disease

The standard treatment for Addison's disease consists of lifelong hormone replacement therapy with oral hydrocortisone (15-25 mg daily in 2-3 divided doses) and fludrocortisone (50-200 μg once daily), with dose adjustments during illness, stress, or surgery to prevent potentially fatal adrenal crisis. 1

Glucocorticoid Replacement

• Hydrocortisone is the first-line glucocorticoid replacement, administered in divided doses (typically 2-3 times daily) to mimic the natural cortisol rhythm, with the first dose immediately after waking and the last dose at least 6 hours before bedtime 1
• The recommended starting dose is 15-25 mg of hydrocortisone daily, with the lowest effective dose being used to minimize side effects while maintaining well-being 1
• In children, hydrocortisone dosing should be 6-10 mg/m² of body surface area 1
• Alternative glucocorticoids like cortisone acetate can be used, but synthetic glucocorticoids may have less favorable metabolic profiles for long-term treatment 2

Mineralocorticoid Replacement

• Fludrocortisone is administered at a dose of 50-200 μg (0.05-0.2 mg) once daily to replace aldosterone 1, 3
• Children and younger adults may require higher doses of fludrocortisone 1
• If essential hypertension develops, the fludrocortisone dose should be reduced but not completely discontinued 1
• Patients should be advised to consume salt and salty foods freely and to avoid licorice and grapefruit juice 1

Management of Adrenal Crisis

• Adrenal crisis requires immediate treatment with 100 mg IV or IM hydrocortisone, followed by 100 mg every 6-8 hours until recovery 1, 4
• Isotonic (0.9%) saline should be administered at an initial rate of 1 L/hour until hemodynamic improvement, with 3-4 L typically given over 24-48 hours 1, 4
• The underlying precipitant of the crisis (e.g., infection) must be identified and treated 1, 4

Special Situations Requiring Dose Adjustments

• During minor illness or stress, patients should double or triple their oral glucocorticoid dose 4
• Surgery and invasive medical procedures require IV or IM hydrocortisone and increased oral doses, with major surgery requiring 100 mg hydrocortisone IM before anesthesia 1, 4
• During pregnancy, small adjustments to hydrocortisone and fludrocortisone doses may be needed, particularly in the third trimester, with parenteral hydrocortisone during delivery 1, 4
• Unaccustomed intense or prolonged exercise may require increased hydrocortisone and salt intake 4

Patient Education and Follow-up

• All patients should receive education on dose adjustments during illness, use of emergency injectable hydrocortisone, and when to seek medical attention 4, 5
• Patients should wear medical alert identification (bracelet/necklace) and carry an emergency steroid card 4, 5
• Patients should be reviewed at least annually, with assessment of general health, weight, blood pressure, and serum electrolytes 1
• Monitoring for the development of new autoimmune disorders, particularly hypothyroidism, is recommended 1
• Bone mineral density should be assessed every 3-5 years to monitor for complications of glucocorticoid therapy 1

Adrenal Androgen Replacement

• There is insufficient evidence to recommend routine replacement of adrenal androgens (DHEA) in all patients with Addison's disease 1, 2
• DHEA replacement may benefit selected patients but remains controversial 2, 6