What is the treatment for Addison's disease?

Treatment for Addison's Disease

The definitive treatment for Addison's disease requires lifelong hormone replacement therapy with both glucocorticoids and mineralocorticoids to prevent potentially fatal adrenal crisis. 1

Glucocorticoid Replacement

• Hydrocortisone (HC) or cortisone acetate (CA) are the preferred glucocorticoids for replacement therapy 1
• Standard dosing for hydrocortisone is 15-25 mg per day divided into 2-3 doses 1, 2
• The first dose should be taken immediately upon waking and the last dose at least 6 hours before bedtime to mimic the natural circadian rhythm 3
• Dosage should be adjusted based on clinical assessment rather than laboratory values 1
• For children, the recommended dosage is 6-10 mg/m² of body surface area 3

Mineralocorticoid Replacement

• Fludrocortisone is the standard mineralocorticoid replacement at a usual dose of 0.1 mg daily 1, 4
• Dosage may range from 0.1 mg three times weekly to 0.2 mg daily, depending on individual response 4
• If transient hypertension develops, the dose should be reduced to 0.05 mg daily 4
• Young adults and children may require higher doses 3
• Fludrocortisone should be administered in conjunction with the glucocorticoid replacement 4

Dose Adjustments for Special Circumstances

Stress Dosing

• During minor illness (fever, infection):
  • Double or triple the usual glucocorticoid dose 1
  • Return to normal dosing once recovered 1

Adrenal Crisis Management

• Adrenal crisis is a life-threatening emergency requiring immediate treatment 1
• Treatment protocol:
  • Immediate administration of hydrocortisone 100 mg IV or IM 1, 3
  • Continue with 100 mg every 6-8 hours until recovery 1, 3
  • Rapid infusion of isotonic (0.9%) sodium chloride solution at 1 L/hour until hemodynamic improvement 1

Pregnancy

• During the third trimester, hydrocortisone dose should be gradually increased by 2.5-10 mg daily 3

Patient Education and Follow-up

• All patients must wear a medical alert bracelet/necklace and carry a steroid emergency card 1, 3

• Patients need comprehensive education on:

  • Recognizing signs of under and over-replacement 1
  • Adjusting medication during illness or stress 1, 3
  • Using emergency injectable hydrocortisone when needed 3
  • When to seek immediate medical attention 3

• Annual follow-up should include:

  • Assessment of overall health and well-being 1, 3
  • Measurement of weight and blood pressure 1, 3
  • Monitoring of serum electrolytes 1, 3
  • Screening for development of other autoimmune disorders, particularly hypothyroidism 1, 3
  • Assessment for complications of glucocorticoid therapy 1

Emerging Treatment Options

• Timed-release hydrocortisone tablets and continuous subcutaneous hydrocortisone infusion are promising new treatment modalities that may better mimic physiological cortisol patterns 2, 5
• These newer approaches may improve quality of life and reduce long-term complications 5

Common Pitfalls and Caveats

• Synthetic glucocorticoids (like prednisone or dexamethasone) may have undesirable metabolic effects with long-term use and are generally not recommended as first-line treatment 2
• Patients often report impaired health-related quality of life despite conventional replacement therapy, suggesting current treatment approaches may still be suboptimal 2, 5
• Many patients with Addison's disease experience fatigue, reduced stress tolerance, and general malaise despite standard therapy 6
• Failure to adequately educate patients about stress dosing is a major risk factor for adrenal crisis 1, 3
• Adrenal androgen replacement (DHEA) has shown inconsistent benefits and is not routinely recommended 2, 6