Addison's disease medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] ; Associate Editor(s)-in-Chief: Aditya Ganti M.B.B.S. [2]

Medical therapy

The mainstay of treatment for Addison's disease is pharmacotherapy which is replacement of deficient hormones. Medical management of Addison's disease can be discussed under two categories

• Acute management ( Addison's crisis)
• Chronic management

Acute management

The main stay of treatment includes glucocorticosteroids and supportive therapy

Goals

• Normalization of blood pressure and volume status
• Supplementation of adequate glucocorticoids plus mineralocorticoid.

Supportive therapy

• Maintain airway, breathing, and circulation, and refer immediately to tertiary care center for intravenous corticosteroids.
• If the patient has pre filled syringes (emergency kit) and presents with Addisonian crisis while far from a hospital, an intramuscular injection should be given and the patient transferred to the nearest emergency room for intravenous normal saline and intravenous hydrocortisone.
• Normal saline 0.9% or 5% dextrose in normal saline should be administered to correct hypotension and dehydration.
• It is usually necessary to administer 1 L rapidly and a further 2 to 4 L over the first 24 hours, to correct hypotension.
• Careful monitoring of BP, fluid status, and serum sodium and potassium levels should be maintained.

Pharmocotherapy

• Dexamethasone should be given to patients with suspected Addisonian crisis prior to any laboratory measurements.
• Intravenous hydrocortisone is used to treat Addisonian crisis following dexamethasone.
• In addition, fludrocortisone is needed for mineralocorticoid replacement.

Adult

• Preferred regimen (1): Dexamethasone IV 2-8 mg/dose q12h followed by a Oral 0.5 mg maintenance dose.
• Preferred regimen (1): Hydrocortisone 100 mg bolus immediately; followed by either 100 mg q8h (or) 300 mg q24 by continuous infusion for 2 to 3 days; then 100 to 150 mg q24h and taper to 75 mg/d before switching to oral maintenance dose
  • Note: Maintenance dose 10 mg in the morning, 5 mg around noon, and 5 mg in the afternoon (or) 10 to 15 mg in the morning and 5 to 10 mg in the afternoon.

Pediatric

Preferred regimen (1): Hydrocortisone 1 to 2 mg/kg/dose bolus immediately; followed by 25 to 150 mg/d, given in divided doses every 6 to 8 hours (in infants and young children)or150 to 250 mg/d given in divided doses every 6 to 8 hours (in older children).

Chronic management

The main stay of treatment includes glucocorticosteroids and mineralocorticoids.

Goals

• Adequate daily supplementation of glucocorticoid and mineralocorticoid to mimic normal physiology. This should aim to maintain normal blood pressure, blood glucose, and fluid volume, and instill a sense of well-being in the patient
• Advise patients on medication for minor illness (febrile illness or emesis) to double or triple their usual dose of glucocorticoid. In case of severe illness, they should inject themselves with a large dose of glucocorticoid and seek immediate medical attention
• If patients are monitored to normalize ACTH level, they are almost invariably overtreated with glucocorticoid resulting in iatrogenic Cushing syndrome. Monitoring is primarily based on clinical features
• Ensure that patients are aware that they must be vigilant in maintaining their therapeutic regimen

Precautions

• All patients with known Addison disease should have an emergency plan in place for corticosteroid supplementation (oral or intramuscular), to be implemented if significant illness occurs
• Immediate action is needed for the signs of Addisonian crisis in a known Addison disease patient
• If the patient has pre filled syringes (emergency kit) and presents with Addisonian crisis while far from a hospital, an intramuscular injection should be given and the patient transferred to the nearest emergency room for intravenous normal saline and intravenous hydrocortisone.
• In an undiagnosed patient who requires immediate corticosteroid treatment, dexamethasone may be given as it does not interfere with ACTH stimulation testing.

Pharmacotherapy

Glucocorticosteroid

• Preferred regimen (1): cortisone 10 to 37.5 mg q12h orally given in 2 divided doses with two-thirds of the total dose given in the morning (around 8 a.m.) and one third in the afternoon (noon to 4 p.m.) or
• Preferred regimen (2): hydrocortisone : 15-30 mg/day orally given in 2 divided doses with two-thirds of the total dose given in the morning (around 8 a.m.) and one third in the afternoon (noon to 4 p.m.) or
• Preferred regimen (3): dexamethasone : 0.25 to 0.75 mg orally once daily
• Preferred regimen (4): prednisone : 2.5 to 5 mg orally once daily

Mineralocorticosteroid'

• Preferred regimen (1): fludrocortisone : 0.1 to 0.2 mg orally once daily

mild-to-moderate stress:

• Alternative regimen (1): cortisone 50-100 mg/day orally given in 2 divided doses with two-thirds of the total dose given in the morning (around 8 a.m.) and one third in the afternoon (noon to 4 p.m.) for 3 days
• Alternative regimen (2): hydrocortisone 30-90 mg/day orally given in 2 divided doses with two-thirds of the total dose given in the morning (around 8 a.m.) and one third in the afternoon (noon to 4 p.m.) for 3 days
• Alternative regimen (3): dexamethasone 0.50 to 2.25 mg orally once daily for 3 days
• Alternative regimen (4): prednisone 5-15 mg orally once daily for 3 days

Severe stress

• Alternative regimen (5): hydrocortisone sodium succinate 100 mg intravenously every 6-8 hours

Women with decreased libido

Androgen replacement

• The ovaries and the adrenals are the main source of androgens in women.
• The adrenals produce dehydroepiandrosterone (DHEA) and its sulfate, which are converted peripherally to androstenedione and testosterone.
• Women with complaints of decreased libido or sexual well-being may be treated with DHEA replacement.
• DHEA should be discontinued periodically to assess these symptoms.

• Preferred regimen (1): DHEA 50 mg orally once daily