What is the treatment for Addison's disease?

Treatment for Addison's Disease

The standard treatment for Addison's disease consists of glucocorticoid replacement with hydrocortisone 15-25 mg daily in divided doses, combined with mineralocorticoid replacement using fludrocortisone 0.05-0.1 mg daily. 1, 2

Pharmacological Management

Glucocorticoid Replacement

• First-line therapy: Hydrocortisone 15-25 mg daily in divided doses (typically 2-3 doses per day) 1
  • Morning dose: Largest portion (typically 50-60% of total daily dose)
  • Afternoon dose: Smaller portion (typically 30-40%)
  • Evening dose (if using 3 doses): Smallest portion (typically 10-20%)
• Synthetic glucocorticoids (prednisolone, dexamethasone) are less preferred due to potential undesirable long-term metabolic effects 3

Mineralocorticoid Replacement

• Fludrocortisone 0.05-0.1 mg once daily 1, 2
• Dosage may range from 0.1 mg three times weekly to 0.2 mg daily 2
• If transient hypertension develops, reduce dose to 0.05 mg daily 2

Stress Dosing Protocol

Patients must follow a stress dosing protocol to prevent adrenal crisis during periods of illness or stress:

1. Minor illness/stress (e.g., cold, low-grade fever):

   • Double or triple usual daily glucocorticoid dose 1

2. Moderate stress (e.g., infection with high fever):

   • Hydrocortisone 50-75 mg/day in divided doses 1

3. Severe stress (e.g., major surgery, trauma, severe illness):

   • Hydrocortisone 100 mg IV immediately 1
   • Followed by 100-300 mg/day as continuous infusion or divided doses every 6 hours 1

4. Post-surgical management:

   • After major surgery: Double oral glucocorticoid doses for 48 hours or up to a week if recovery is complicated 1
   • After minor procedures: Double oral glucocorticoid doses for 24 hours, then return to normal maintenance dose 1

Monitoring and Follow-up

Regular monitoring should include:

• Overall well-being and quality of life
• Weight and blood pressure
• Serum electrolytes (particularly sodium and potassium) 1
• Signs of over-replacement (weight gain, hypertension, edema)
• Signs of under-replacement (fatigue, weight loss, hypotension)

Patient Education - Critical Components

All patients with Addison's disease must receive education on:

• Proper administration of daily medications
• Stress dosing protocols for illness
• Use of emergency injectable steroids
• When to seek medical attention
• Importance of wearing medical alert identification 1

Special Considerations

• Adrenal crisis management: Requires immediate administration of hydrocortisone 100 mg IV and fluid resuscitation 1
• Comorbidities: Patients with asthma and diabetes require closer monitoring due to increased vulnerability to adrenal crisis 1
• Never abruptly discontinue steroids: Always taper to prevent rebound adrenal insufficiency 1

Emerging Therapies

Recent developments in treatment options include:

• Timed-release hydrocortisone tablets to better mimic physiological cortisol rhythm 3, 4
• Continuous subcutaneous hydrocortisone infusion for selected patients 3, 4

Common Pitfalls to Avoid

1. Delayed diagnosis: Due to non-specific symptoms, diagnosis is often delayed 5, 6
2. Inadequate stress dosing education: Leading cause of preventable adrenal crises
3. Inappropriate glucocorticoid dosing: Both over-replacement and under-replacement have significant consequences
4. Failure to recognize adrenal crisis: Requires immediate intervention to prevent mortality

The mortality rate in patients with Addison's disease remains elevated despite treatment, highlighting the importance of proper management and patient education 6.