What is the treatment for adrenal insufficiency?

Treatment of Adrenal Insufficiency

The standard treatment for adrenal insufficiency consists of hydrocortisone 15-25 mg daily in divided doses (with the first dose upon waking) and fludrocortisone 50-200 μg daily as a single dose for patients with primary adrenal insufficiency. 1

Glucocorticoid Replacement

Daily Maintenance Therapy

• Hydrocortisone: First-line treatment
  • Dosage: 15-25 mg daily in divided doses 1, 2
  • Typical regimens:
    • Three doses: 10 mg (morning) + 5 mg (noon) + 2.5 mg (afternoon)
    • Two doses: 15 mg (morning) + 5 mg (noon) 1
  • For children: 6-10 mg/m² of body surface area daily 1, 3
• Cortisone acetate: Alternative option
  • Dosage: 25-37.5 mg daily in divided doses 1
• Prednisolone: Consider only if compliance issues or when hydrocortisone is not tolerated
  • Dosage: 4-5 mg daily 1

Administration Schedule

• First dose immediately after waking
• Last dose not less than 6 hours before bedtime to avoid sleep disturbances 1
• Use the lowest effective dose that maintains health and well-being 1

Mineralocorticoid Replacement

• Fludrocortisone: Required for primary adrenal insufficiency only

  • Dosage: 50-200 μg as a single daily dose upon awakening 1, 4, 2
  • Children and younger adults may require higher doses (up to 500 μg daily) 1
  • Not required for secondary adrenal insufficiency 2

• Dietary recommendations:

  • Unrestricted salt and salty food intake
  • Avoid potassium-containing salt substitutes 1
  • Avoid liquorice and grapefruit juice (potentiate mineralocorticoid effects) 1

Management During Special Circumstances

Adrenal Crisis Management

1. Immediate administration of hydrocortisone: 100 mg IV/IM followed by 100 mg every 6-8 hours until recovery 1, 3
2. Rapid infusion of isotonic (0.9%) saline: Initial rate of 1 L/hour until hemodynamic improvement 1
3. Identify and treat the underlying precipitant (e.g., infection) 1

Surgery and Medical Procedures

• Increase steroid doses according to degree of stress
• For major surgery: 100 mg hydrocortisone IV/IM before procedure, followed by regular doses during recovery 1

Pregnancy

• May require increased hydrocortisone dose by 2.5-10 mg daily in third trimester
• Fludrocortisone dose often needs to be increased during late pregnancy due to progesterone's anti-mineralocorticoid effects
• During delivery: 100 mg parenteral hydrocortisone, repeated every 6 hours if necessary
• Double oral dose for 24-48 hours postpartum 1

Physical Activity

• Regular, accustomed activity: No dose adjustment needed
• Intense or prolonged exercise: Consider extra 5 mg hydrocortisone before activity
• In hot conditions: Additional fluid and salt intake 1

Adrenal Androgen Replacement

• Not routinely recommended due to insufficient evidence of benefit 1
• Consider for female patients with persistent lack of libido/energy despite optimized glucocorticoid and mineralocorticoid replacement
• DHEA 25 mg daily as a trial for 6 months, continue if clinically effective 1, 2

Patient Education and Follow-up

Patient Education

• All patients should:
  • Wear medical alert identification
  • Carry a steroid/alert card
  • Receive education on managing daily medications and minor illnesses
  • Have supplies for self-injection of parenteral hydrocortisone 1, 3

Follow-up

• Annual review with assessment of:
  • Health and well-being
  • Weight and blood pressure
  • Serum electrolytes
  • Development of new autoimmune disorders (particularly hypothyroidism)
  • Bone mineral density every 3-5 years 1

Medication Interactions

Drugs Affecting Hydrocortisone

• May need increased dose with:
  • Anti-epilepsy medications/barbiturates
  • Antituberculosis drugs
  • Etomidate
  • Topiramate 1

Drugs Affecting Fludrocortisone

• Avoid:
  • Diuretics
  • Acetazolamide
  • NSAIDs
  • Carbenoxolone 1

Common Pitfalls and Caveats

1. Delayed diagnosis: Adrenal insufficiency often presents with non-specific symptoms (fatigue, nausea, weight loss) leading to delayed recognition 2, 5

2. Undertreating mineralocorticoid deficiency: Under-replacement is common and sometimes compensated by over-replacement of glucocorticoids 1

3. Failure to adjust doses during stress: Inadequate glucocorticoid therapy during acute illness or physical stress can precipitate life-threatening adrenal crisis 2, 5

4. Essential hypertension management: If a patient develops essential hypertension, reduce fludrocortisone dose but do not stop it completely; add a vasodilator if needed 1

5. Inadequate patient education: Lack of proper education about stress dosing and emergency management increases risk of adrenal crisis 5, 6