What is the treatment for adrenal insufficiency?

Treatment for Adrenal Insufficiency

The standard treatment for adrenal insufficiency consists of glucocorticoid replacement with hydrocortisone (15-25 mg daily in divided doses) and mineralocorticoid replacement with fludrocortisone (0.05-0.2 mg daily) for primary adrenal insufficiency. 1

Diagnosis and Classification

Before initiating treatment, it's important to establish the type of adrenal insufficiency:

• Primary adrenal insufficiency: Characterized by low morning cortisol and high ACTH levels, often with hyponatremia, hyperkalemia, and hyperpigmentation
• Secondary adrenal insufficiency: Low cortisol with low or inappropriate ACTH levels
• Tertiary adrenal insufficiency: Due to exogenous glucocorticoid therapy

Diagnostic tests include:

• Morning serum cortisol and ACTH levels
• ACTH stimulation test (for equivocal cases)
• Electrolytes (Na, K, glucose)

Treatment Algorithm

1. Maintenance Therapy for Primary Adrenal Insufficiency

• Glucocorticoid replacement:

  • Hydrocortisone 15-25 mg daily in divided doses (typically 2-3 doses)
  • First dose immediately after waking, last dose at least 6 hours before bedtime
  • In children: 6-10 mg/m² of body surface area 1

• Mineralocorticoid replacement:

  • Fludrocortisone 0.05-0.2 mg once daily 1, 2
  • Adjust based on blood pressure, serum electrolytes, and plasma renin activity
  • Not required in secondary adrenal insufficiency

2. Maintenance Therapy for Secondary Adrenal Insufficiency

• Glucocorticoid replacement only:
  • Same hydrocortisone dosing as primary insufficiency
  • No mineralocorticoid replacement needed (aldosterone production intact)

3. Acute Adrenal Crisis Management

• Immediate treatment:

  • Hydrocortisone 100 mg IV bolus, followed by 100-300 mg/day as continuous infusion or divided doses every 6 hours 1
  • Rapid IV isotonic saline (0.9%) at initial rate of 1 L/hour 1
  • Treat precipitating causes (infection, etc.)

• Tapering after crisis:

  • Taper parenteral glucocorticoids over 1-3 days to oral maintenance therapy
  • Restart fludrocortisone when hydrocortisone dose falls below 50 mg/day 1

4. Stress Dosing for Illness, Surgery, or Procedures

• Minor illness/stress (fever, minor infection):

  • Double or triple usual oral glucocorticoid dose
  • Return to normal dose when recovered

• Major stress/surgery:

  • Hydrocortisone 100 mg IV at start of surgery
  • Continue with 200 mg/24 hours as infusion
  • For uncomplicated recovery: double regular oral dose for 48 hours up to a week 1

Patient Education and Crisis Prevention

All patients must:

• Wear medical alert identification (bracelet/card)
• Receive education on daily medication management
• Learn stress dosing for illness
• Have emergency injectable hydrocortisone available
• Understand when to seek medical help

Monitoring and Follow-up

• Annual review including:
  • Assessment of well-being and quality of life
  • Weight and blood pressure measurement
  • Serum electrolytes
  • Screening for other autoimmune disorders (especially thyroid dysfunction)
  • Bone mineral density every 3-5 years 1

Common Pitfalls to Avoid

1. Delayed treatment of suspected adrenal crisis - Treatment should never be delayed for diagnostic procedures 1

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

3. Overlooking mineralocorticoid replacement in primary adrenal insufficiency

4. Starting thyroid hormone replacement before glucocorticoids in patients with multiple hormone deficiencies - This can precipitate adrenal crisis 1

5. Medication errors during hospitalization - A significant cause of adrenal crises in hospitalized patients 1

The goal of treatment is to restore normal quality of life and prevent adrenal crises, which occur at a rate of 6-8 per 100 patient-years and can be life-threatening if not promptly treated 1, 3.