What is the treatment for Addison's disease?

Treatment for Addison's Disease

The treatment for Addison's disease requires lifelong dual hormone replacement with oral hydrocortisone 15-25 mg daily (divided into 2-3 doses) plus fludrocortisone 50-200 μg once daily, with mandatory patient education on stress dosing to prevent fatal adrenal crisis. 1

Glucocorticoid Replacement Therapy

Hydrocortisone is the first-line glucocorticoid, administered in divided doses to mimic natural cortisol rhythm. 1

• Start with 15-25 mg hydrocortisone daily, divided into 2-3 doses, with the first dose immediately upon waking and the last dose at least 6 hours before bedtime. 1
• The dosing mimics physiological cortisol secretion patterns, which cannot be achieved with once-daily synthetic glucocorticoids. 1
• In children, dose hydrocortisone at 6-10 mg/m² of body surface area. 1
• Use the lowest effective dose to minimize long-term side effects while maintaining patient well-being. 1

Alternative glucocorticoid: Cortisone acetate 18.75-31.25 mg daily in divided doses can be used, though hydrocortisone remains preferred. 2

Mineralocorticoid Replacement Therapy

Fludrocortisone is essential for aldosterone replacement and must never be completely discontinued. 1, 2

• Standard dosing is 50-200 μg (0.05-0.2 mg) once daily, as confirmed by FDA labeling. 3
• The FDA-approved usual dose is 0.1 mg daily, with a range from 0.1 mg three times weekly to 0.2 mg daily. 3
• Children and younger adults typically require higher doses within this range. 1, 2
• Titrate dose based on blood pressure, serum electrolytes (sodium/potassium), and salt cravings—NOT plasma renin activity. 2

Monitoring parameters: 2

• Check blood pressure at each visit; postural hypotension indicates insufficient replacement or inadequate salt intake
• Measure serum electrolytes at least annually to detect hyponatremia or hyperkalemia
• If essential hypertension develops, reduce fludrocortisone dose but add a vasodilator antihypertensive rather than stopping mineralocorticoid replacement

Dietary guidance: Patients should consume salt and salty foods freely according to taste, and avoid licorice and grapefruit juice. 1, 2

Management of Adrenal Crisis

Adrenal crisis is a medical emergency requiring immediate treatment without waiting for diagnostic confirmation. 4

Immediate treatment protocol: 1, 4

• Administer hydrocortisone 100 mg IV bolus immediately
• Begin fluid resuscitation with 0.9% saline 1 L over the first hour
• Continue with 3-4 L of isotonic saline over 24-48 hours until hemodynamic improvement
• Continue hydrocortisone 100 mg IV/IM every 6-8 hours (or 100-300 mg/day as continuous infusion) until recovery
• Identify and treat the underlying precipitant (infection, trauma, surgery)

Stress Dosing Protocols

Minor illness or stress: Double or triple the oral glucocorticoid dose. 1, 4

Major surgery: 1, 4

• Administer 100 mg hydrocortisone IM before anesthesia
• Continue 100 mg every 6 hours until able to resume oral medication
• Follow stress dosing protocols based on procedure severity

Pregnancy: 1, 4

• Small adjustments to hydrocortisone and fludrocortisone may be needed, particularly in the third trimester
• Administer 100 mg hydrocortisone IM at onset of labor
• Increase fludrocortisone dose during late pregnancy due to progesterone's anti-mineralocorticoid effects

Intense or prolonged exercise: May require increased hydrocortisone and salt intake. 1, 4

Critical Patient Education and Safety Measures

Every patient must receive comprehensive education and emergency supplies: 1, 4

• Wear medical alert identification (bracelet/necklace) at all times 1, 4
• Carry an emergency steroid card 1, 2
• Have emergency injectable hydrocortisone available 4
• Understand when to double/triple doses during illness
• Know when to seek immediate medical attention
• Understand how to self-administer emergency injectable hydrocortisone

Long-Term Monitoring

Annual review should include: 1

• Assessment of general health, weight, and blood pressure
• Serum electrolytes measurement
• Screening for new autoimmune disorders, particularly hypothyroidism
• Bone mineral density assessment every 3-5 years to monitor for glucocorticoid-related complications

Special consideration during high-dose glucocorticoid therapy: When hydrocortisone doses exceed 50 mg daily (such as during acute illness), fludrocortisone can be temporarily held and restarted only when the hydrocortisone dose falls below 50 mg daily, as high-dose hydrocortisone provides sufficient mineralocorticoid activity. 2