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{{Addison's disease}}
{{Addison's disease}}


{{CMG}}
{{CMG}} ; {{AE}} {{ADG}}
==Overview==


==Medical Therapy==
=== Maintenance treatment===
Treatment for Addison's disease involves replacing the missing cortisol (usually in the form of [[hydrocortisone]] tablets) in a dosing regimen that mimics the physiological concentrations of cortisol. Treatment must usually be continued for life. In addition, many patients require [[fludrocortisone]] as replacement for the missing aldosterone. Caution must be exercised when the person with Addison's disease becomes unwell, has [[surgery]] or becomes [[pregnant]]. Medication may need to be increased during times of stress, infection, or injury.


===Addisonian crisis===
==Medical therapy==
Treatment for an acute attack, an Addisonian crisis, usually involves intravenous (into blood veins) injections of:
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
* Cortisone ([[cortisol]])
*Acute management ( Addison's crisis)
* Saline solution (basically a salt water, same clear [[Intravenous therapy#Infusion equipment|IV bag]] as used to treat dehydration)
*Chronic management 
* [[Glucose]]
===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.


=== Pregnancy ===
===Pharmocotherapy===
Many women with Addison's have given birth successfully and without complication, both through natural labor and through cesarean delivery. Both of these methods will require different preventative measures relating to Addison's medications and dosages. Thorough communication with one's primary care physician is the best course of action. Occasionally, oral intake of medications will cause debilitating nausea and vomiting, and thus the woman may be switched to injected medications until delivery. Addison's treatments are generally considered safe for the baby during pregnancy.
*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.
====Contraindicated medications====
*In addition, fludrocortisone is needed for mineralocorticoid replacement.
 
====Adult====
{{MedCondContrAbs
:*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
|MedCond = Addison's disease|Spironolactone|Hydrochlorothiazide}}
:**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


==References==
==References==

Revision as of 06:15, 15 August 2017

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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]

Overview

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

References

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