Adrenal Insufficiency (Addison Disease)

Description

General Measures

Medicine Treatment

Referral

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ICD-10 Code: E27.1/E27.2

DESCRIPTION

Primary adrenocortical insufficiency.

Clinical presentation

Acute crisis: (not all symptoms and signs may occur in a particular patient, so a high index of suspicion is needed).

• hypotension
• depressed mentation
• fever
• hypoglycaemia
• GIT disturbances
• hyponatremia
• dehydration
• hyperkalaemia
• weakness
• acidosis

Chronic:

• hyperpigmentation
• GIT disturbances
• weakness and fatigue
• hypotension
• loss of weight
• hypoglycaemia
• postural dizziness
• arthralgia
• hyponatraemia
• hyperkalaemia

Always consider this diagnosis in a thin, hypotensive, hypoglycaemic patient, or during stress e.g. sepsis. The combination of hyponatraemia and hyperkalaemia should suggest possible primary adrenal insufficiency.

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Note
Treatment of suspected acute adrenal failure should never be delayed to obtain results of diagnostic procedures.

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Investigations

• 08h00 serum cortisol level (or at time of presentation in acute crisis):
  • >500 nmol/L: virtually exclues the diagnosis
  • with newer assays cortisol concentrations >450 nmol/L are acceptable to exclude hypoadrenalism
  • 100-450 nmol/L is indeterminate and may require an adrenocorticotropic hormone (ACTH) stimulation test:
• ACTH depot, IM, 1 mg with blood sampling at 60 minutes
  • Post ACTH, serum cortisol level normal value: >550 nmol/L or double the pre-test level.

LoE:III¹

GENERAL MEASURES

• All patients with confirmed hypoadrenalism.
• Investigate for other causes such as sepsis and treat accordingly.

MEDICINE TREATMENT

Acute crisis

ICD-10 Code: E27.2

Before administering hydrocortisone, ensure blood samples are taken for serum cortisol and plasma ACTH, if feasible.

• Hydrocortisone, IV,100 mg 6 hourly.

  • Change to oral maintenance therapy once stable.

LoE:III²

To maintain adequate intravascular volume guided by blood pressure:

• Sodium chloride 0.9%, IV with regular glucose monitoring, and 50% dextrose boluses if required.
  • Beware of fluid overload if the combination of sodium chloride 0.9%/dextrose 5% is utilised.
  • The fluid deficit is often several litres.

LoE:III³

Monitor glucose levels closely and treat hypoglycaemia if present.

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Note
All suspected cases should be referred for full evaluation, prior to chronic maintenance therapy.

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Chronic

As maintenance therapy:

• Hydrocortisone, oral.
  • Start with 10 mg in the morning and 5 mg at night.
  • Increase the dose according to clinical response up to 20 mg in the morning and 10 mg at night.
  • In patients requiring a midday dose, a suggested regimen is 10 mg in the morning, 5 mg at midday and 5 mg in the early evening

LoE:III

OR

• Corticosteroids (intermediate-acting) e.g.:
  • Prednisone, oral.
    • Start with 5 mg daily.
    • Increase to maximum of 7.5 mg daily, if necessary.

LoE:III⁴

For patients who have symptoms of mineralocorticoid deficiency:

• Fludrocortisone, oral, 50–100 mcg daily may be required to normalise the potassium and to reduce postural hypotension in primary hypoadrenalism.

  • Titrate dose of fludrocortisone in consultation with a specialist.

LoE:III⁵

Monitor response to therapy with:

• Symptoms: improvement in fatigue and GIT disturbances.
• Blood pressure: normotensive and no postural drop.
• Electrolytes: normal Na+ and K+.

During times of severe “stress” i.e. acute illness, surgery, trauma, etc.:

• Hydrocortisone, IV, 100 mg 6 hourly.

LoE:III⁶

Minor stressors e.g.: Influenza, diarrhoeal illness, chest infections and dental procedures warrant doubling of the doses of hyrdrocortisone for the duration of illness and gradual tapering back to usual dose.

REFERRAL

• All suspected cases, for full evaluation.

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Standard Treatment Guidelines and Essential Medicines List for South Africa, Hospital Level, Adults 2024 Edition