What is the maintenance dose and frequency for hydrocortisone in Addison's disease?

Hydrocortisone Maintenance Dosing in Addison's Disease

The standard maintenance dose for hydrocortisone in Addison's disease is 15-25 mg daily, divided into 2-3 doses, with the first dose immediately after waking and the last dose no less than 6 hours before bedtime. 1

Glucocorticoid Replacement Regimen

• Hydrocortisone is the preferred glucocorticoid for replacement therapy in Addisonian disease 1, 2
• The total daily dose should be 15-25 mg for adults, divided into multiple doses to mimic the natural cortisol rhythm 1, 2
• Common dosing schedules include:
  • Three-dose regimen: 10 mg on waking, 5 mg at noon, 2.5-5 mg in late afternoon 2
  • Two-dose regimen: 2/3 of the dose in the morning upon waking and 1/3 in early afternoon 1
• The first dose should be taken immediately after waking, and the last dose should be taken at least 6 hours before bedtime to avoid sleep disturbances 1
• For children, the recommended dose is 6-10 mg/m² of body surface area 1

Mineralocorticoid Replacement

• Most patients with primary adrenal insufficiency also require mineralocorticoid replacement with fludrocortisone 50-200 μg (0.05-0.2 mg) once daily 1, 2
• Children and younger adults may require higher doses of fludrocortisone 1
• Fludrocortisone is typically administered as a single morning dose 2

Dose Adjustments and Monitoring

• The lowest dose compatible with health and a sense of well-being should be used 1
• Signs of over-replacement include weight gain, insomnia, and peripheral edema 1
• Signs of under-replacement include lethargy, nausea, poor appetite, weight loss, and increased pigmentation 1
• Patients should be reviewed at least annually, with assessment of health and well-being, weight, blood pressure, and serum electrolytes 1
• Bone mineral density should be monitored every 3-5 years to assess for complications of glucocorticoid therapy 2

Special Situations

Stress Dosing

• During minor to moderate illness, patients should increase their glucocorticoid dose 2-3 times 3, 2
• For surgery or invasive medical procedures, intravenous or intramuscular hydrocortisone and increased oral doses are required 1, 2
• In adrenal crisis, immediate treatment with 100 mg IV or IM hydrocortisone is needed, followed by 100 mg every 6-8 hours until recovery 1, 3

Pregnancy

• Small adjustments to hydrocortisone and fludrocortisone doses may be needed during pregnancy, particularly during the last trimester 1
• Parenteral doses of hydrocortisone should be given during delivery 1

Patient Education

• All patients should wear medical alert identification and carry a steroid alert card 1, 2
• Patients should be provided with supplies to allow self-injection of parenteral hydrocortisone 1, 2
• Education on stress dosing during illness or injury is essential 1, 3

Common Pitfalls

• Under-replacement with mineralocorticoids is common and sometimes compensated for by over-replacement with glucocorticoids 2
• Drugs that may increase hydrocortisone requirements include anti-epileptics, antituberculosis medications, and etomidate 2
• Drugs that may decrease hydrocortisone requirements include grapefruit juice and licorice 2
• NSAIDs should be avoided with fludrocortisone 2

By following these dosing guidelines and monitoring parameters, patients with Addison's disease can maintain adequate hormone replacement and minimize complications associated with both over- and under-replacement.