What is the treatment for Addison's disease?

Treatment for Addison's Disease

The definitive treatment for Addison's disease consists of lifelong hormone replacement therapy with hydrocortisone 15-25 mg daily in divided doses and fludrocortisone 0.05-0.1 mg daily. 1

Standard Maintenance Therapy

Glucocorticoid Replacement

• 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%)
• This divided dosing schedule better mimics the natural cortisol rhythm

Mineralocorticoid Replacement

• Fludrocortisone 0.05-0.1 mg once daily 1, 2
• FDA-approved dosage range: 0.1 mg daily is standard, though some patients may require:
  • As little as 0.1 mg three times weekly
  • As much as 0.2 mg daily 2
• Dosage should be reduced to 0.05 mg daily if transient hypertension develops 2

Stress Dosing Protocol

Patients with Addison's disease require increased glucocorticoid doses during periods of stress to prevent adrenal crisis:

1. Minor illness/stress (e.g., cold, mild fever):

   • Double or triple the usual daily hydrocortisone 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
   • Follow with 100-300 mg/day as continuous infusion or divided doses every 6 hours 1

Management of Adrenal Crisis

Adrenal crisis is a life-threatening emergency requiring immediate treatment:

1. Immediate intervention:

   • Administer 100 mg hydrocortisone IV immediately 1
   • Continue hydrocortisone 100 mg IV every 6 hours until stabilization 1
   • Provide aggressive fluid resuscitation with normal saline

2. Transition to oral therapy:

   • Once the patient improves and can tolerate oral intake, transition to oral hydrocortisone
   • Double the usual maintenance dose for 24-48 hours 1
   • Gradually taper to maintenance dose (never abruptly discontinue)

Post-Surgical Management

1. After major surgery:

   • Double the pre-surgical glucocorticoid dose for 48 hours
   • Continue double dose for up to a week if recovery is complicated 1

2. After minor procedures:

   • Double oral glucocorticoid doses for 24 hours
   • Return to normal maintenance dose thereafter 1

Patient Education and Monitoring

Essential Education Points

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

Monitoring Parameters

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

Special Considerations

High-Risk Populations

• Patients with comorbidities (especially asthma and diabetes) require closer monitoring due to increased vulnerability to adrenal crisis 1
• Patients with cirrhosis hepatica have higher rates of relative adrenal insufficiency (approximately 49%) 1

Alternative Therapies

Recent developments in Addison's disease treatment include modified-release hydrocortisone and continuous subcutaneous hydrocortisone infusion, which may better simulate physiological cortisol rhythm 3. However, these are not yet standard of care.

Common Pitfalls and Caveats

1. Delayed diagnosis: The non-specific symptoms of Addison's disease often lead to delayed diagnosis 4, 5. Consider Addison's disease in patients with unexplained fatigue, weight loss, hyperpigmentation, and electrolyte abnormalities.

2. Inadequate stress dosing: Failure to increase glucocorticoid doses during illness or stress is a common cause of adrenal crisis 1.

3. Abrupt discontinuation: Never abruptly discontinue steroids; always taper to prevent rebound adrenal insufficiency 1.

4. Over-replacement: Chronic over-replacement with glucocorticoids can lead to cushingoid features, osteoporosis, and metabolic complications 6.

5. Under-replacement: Insufficient replacement can lead to chronic fatigue, reduced quality of life, and increased risk of adrenal crisis 6.