What is the treatment for Addison's disease?

Treatment for Addison's Disease

Addison's disease requires lifelong hormone replacement with oral hydrocortisone 15-25 mg daily (divided into 2-3 doses) plus fludrocortisone 50-200 μg once daily, with immediate dose escalation during illness or stress to prevent potentially fatal adrenal crisis. 1

Glucocorticoid Replacement Therapy

Hydrocortisone is the first-line glucocorticoid, administered in divided doses to mimic natural cortisol rhythm 1:

• Dosing schedule: Give the first dose immediately upon waking, with subsequent doses throughout the day, ensuring the last dose is taken at least 6 hours before bedtime 1
• Adult dosing: Start with 15-25 mg daily total, divided into 2-3 doses (commonly 10 mg morning, 5 mg midday, 5 mg afternoon) 1
• Pediatric dosing: 6-10 mg/m² of body surface area daily 1
• Use the lowest effective dose that maintains patient well-being to minimize long-term side effects 1

Mineralocorticoid Replacement Therapy

Fludrocortisone replaces aldosterone deficiency 2:

• Standard dose: 50-200 μg (0.05-0.2 mg) once daily 1, 2
• Higher doses may be needed in children and younger adults 1
• If hypertension develops, reduce the dose to 0.05 mg daily but do not discontinue completely 1, 2
• Dietary guidance: Patients should consume salt and salty foods freely, while avoiding licorice and grapefruit juice 1

The FDA label confirms fludrocortisone 0.1 mg daily is the usual dose, with a range of 0.1 mg three times weekly to 0.2 mg daily, preferably combined with hydrocortisone 10-30 mg daily 2.

Management of Adrenal Crisis

Adrenal crisis is a medical emergency requiring immediate treatment without waiting for diagnostic confirmation 3:

Immediate Actions:

• Hydrocortisone 100 mg IV or IM bolus immediately 1, 3
• Isotonic saline (0.9%) at 1 L/hour initially until hemodynamic improvement 1, 3
• Continue hydrocortisone 100 mg every 6-8 hours until recovery 1, 3
• Administer 3-4 L of saline over 24-48 hours total 1, 3
• Identify and treat the precipitating cause (infection, trauma, surgery) 1, 3

Common pitfall: Mineralocorticoid replacement with fludrocortisone should be restarted only when the hydrocortisone dose falls below 50 mg daily, as high-dose hydrocortisone provides sufficient mineralocorticoid activity 4.

Dose Adjustments for Special Situations

Minor Illness or Stress:

• Double or triple the oral glucocorticoid dose during febrile illness, gastroenteritis, or minor infections 1, 3

Major Surgery:

• 100 mg hydrocortisone IM before anesthesia induction 1, 3
• Continue 100 mg every 6 hours until able to resume oral medication 3

Pregnancy:

• Small dose increases (2.5-10 mg additional hydrocortisone daily) may be needed in the third trimester 1, 5
• 100 mg hydrocortisone IM at onset of labor 3, 5
• Fludrocortisone adjustments may also be necessary 1

Intense Exercise:

• Unaccustomed prolonged or intense exercise may require increased hydrocortisone and salt intake 1, 3

Patient Education and Safety Measures

All patients must receive comprehensive education to prevent adrenal crises 4, 1:

• Emergency supplies: Prescribe injectable hydrocortisone for home use 1, 3
• Medical identification: Wear medical alert bracelet/necklace and carry emergency steroid card at all times 1, 3
• Sick day rules: Teach specific protocols for doubling/tripling doses during illness and when to seek emergency care 1, 3
• Empower patients to increase steroid doses during intercurrent illnesses, vomiting, injuries, or other stressors before reaching a state where they cannot care for themselves 4

Annual Follow-Up and Monitoring

Regular medical examinations are essential 4, 1:

• At least annual visits assessing general health, weight, blood pressure, and serum electrolytes (sodium, potassium) 4, 1
• Thyroid function testing (TSH, FT4, TPO antibodies) every 12 months, as hypothyroidism frequently develops 4, 1
• Screen for associated autoimmune conditions: Check plasma glucose, HbA1c, complete blood count, and vitamin B12 levels annually 4, 1
• Bone mineral density assessment every 3-5 years to monitor for glucocorticoid-induced osteoporosis 1
• Consider morning cortisol absorption testing (serum or saliva at 0,2,4, and 6 hours post-dose) if under-replacement is suspected 4

Common pitfall: Low salt consumption and chronic mineralocorticoid under-replacement are causes of recurrent adrenal crises that must be investigated 4.