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 and mineralocorticoid replacement with fludrocortisone 0.05-0.1 mg daily. 1, 2

Hormone Replacement Therapy

Glucocorticoid Replacement

• Hydrocortisone is the first-line glucocorticoid replacement:
  • Dosage: 15-25 mg daily
  • Administration: Divided into 2-3 doses to mimic normal cortisol production
  • Typical schedule: Higher dose in morning (⅔ of daily dose), smaller dose in afternoon/evening
  • Weight-adjusted dosing is preferred for optimal management 3

Mineralocorticoid Replacement

• Fludrocortisone is required for all patients with primary adrenal insufficiency:
  • Standard dosage: 0.05-0.1 mg once daily
  • Dose range: Can vary from 0.05 mg three times weekly to 0.2 mg daily
  • Adjustment: Reduce to 0.05 mg daily if transient hypertension develops 2

Monitoring and Dose Adjustment

Regular Monitoring Parameters

• Clinical assessment:
  • Overall well-being
  • Weight
  • Blood pressure (target: normal range without orthostatic hypotension)
• Laboratory monitoring:
  • Serum electrolytes (sodium, potassium)
  • Plasma renin activity (for mineralocorticoid adequacy)

Follow-up Schedule

• Initial period: Every 1-3 months
• Stable patients: Annual follow-up
• Annual screening for associated autoimmune conditions 1
• Bone mineral density assessment every 3-5 years 1

Stress Dosing Protocol

Patients must be educated about increasing glucocorticoid doses during illness or stress:

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

   • Double or triple usual daily hydrocortisone dose

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

   • Hydrocortisone 50-75 mg/day in divided doses

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

   • Hydrocortisone 100 mg IV immediately
   • Follow with 100-300 mg/day as continuous infusion or divided doses every 6 hours 1, 4

Adrenal Crisis Management

Adrenal crisis requires immediate treatment:

• Hydrocortisone 100 mg IV bolus
• Normal saline infusion for volume resuscitation
• Taper to maintenance dose over 5-10 days after stabilization 1

Additional Considerations

DHEA Replacement

• Consider DHEA replacement (25-50 mg daily) for patients with persistent symptoms despite adequate primary replacement therapy
• Most beneficial for women with low libido or persistent fatigue 1, 3

Emerging Treatment Options

• Timed-release hydrocortisone tablets and continuous subcutaneous hydrocortisone infusion are promising new treatment modalities that better mimic physiological cortisol rhythms 3, 5

Common Pitfalls to Avoid

1. Inadequate stress dosing education:

   • All patients must receive detailed instructions and emergency supplies
   • Patients should have injectable hydrocortisone and know how to use it

2. Over-replacement:

   • Excessive glucocorticoid doses can lead to weight gain, osteoporosis, and metabolic complications
   • Aim for the lowest effective dose that controls symptoms

3. Under-replacement:

   • Insufficient dosing can lead to chronic fatigue, weight loss, and risk of adrenal crisis
   • Monitor for signs of inadequate replacement

4. Relying solely on serum cortisol levels:

   • Timing of the last hydrocortisone dose affects results
   • Clinical assessment remains essential 1

5. Failure to screen for associated autoimmune conditions:

   • Autoimmune Addison's disease often occurs with other autoimmune disorders
   • Regular screening is necessary 1, 6