Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

INITIAL

adrenal crisis

1st line – intravenous hydrocortisone and supportive therapy

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INITIAL
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adrenal crisis
1st line – 

intravenous hydrocortisone and supportive therapy

Intravenous hydrocortisone should be given to patients with a suspected but unconfirmed diagnosis of adrenal insufficiency.[2][26][38][42]

Saline should be administered to correct hypotension and dehydration.[26][38] It is usually necessary to administer 1 L rapidly, and a further 4 to 6 L over the first 24 hours, to correct hypotension.[26] Careful monitoring of blood pressure, fluid status, and serum sodium and potassium levels should be maintained.

Glucose should be administered when necessary to correct hypoglycaemia, but care should be taken to avoid worsening hyponatraemia.[38] The use of normal saline supplemented with dextrose 5% is helpful in this regard.

The underlying cause that precipitated the crisis should be sought and treated, and once the patient is stable, normal dosing regimen can be resumed.

Education for the patient and family members on symptoms and circumstances of risk of adrenal crisis and information on how to manage dose adjustments of glucocorticoid replacement therapy may prevent further episodes.[8] All patients are encouraged to carry a steroid card or medical alert bracelet to inform medical personnel.

Primary options

hydrocortisone sodium succinate: 100 mg intravenously/intramuscularly as a single dose, followed by 200 mg per 24 hours as a continuous infusion (or 50 mg intravenously/intramuscularly every 6 hours), taper dose according to response as patient becomes stable

ACUTE

minor intercurrent stress

1st line – temporary double dose of existing corticosteroid

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ACUTE
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minor intercurrent stress
1st line – 

temporary double dose of existing corticosteroid

Patients with primary adrenal insufficiency experiencing minor intercurrent stress (e.g., febrile illness; minor procedure/surgery not requiring fasting such as tooth extraction or local anaesthesia) should be instructed to double their chronic maintenance dose of corticosteroid on the day of the procedure or for the duration of illness.[2][26]

They then return to the usual dose when the stress resolves.

severe intercurrent stress

1st line – intravenous hydrocortisone (stress dose)

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ACUTE
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severe intercurrent stress
1st line – 

intravenous hydrocortisone (stress dose)

Patients with primary adrenal insufficiency who undergo severe stress situations (e.g., unable to take oral glucocorticoid, such as acute gastroenteritis or prolonged fasting for colonoscopy; surgery under general or regional anaesthesia; critical illness requiring ventilation; major trauma; active phase of labour and delivery) require parenteral corticosteroid (usually hydrocortisone).[2][4][26][45][46] However, evidence for the timing and dosage of perioperative stress-dose glucocorticoids is limited and will benefit from studies comparing different strategies.[46][47][48][49]

If patients are out of the critical phase of illness in less than 1 week but remain ill, then the dose of the corticosteroid can be tapered, using an oral formulation, back to previous pre-illness doses. Consult your local guidelines for a suitable corticosteroid taper regimen.

Primary options

hydrocortisone sodium succinate: 100 mg intravenously/intramuscularly as a single dose, followed by 200 mg per 24 hours as a continuous infusion (or 50 mg intravenously/intramuscularly every 6 hours), taper dose according to response as patient becomes stable

ONGOING

stable and/or after treatment of acute episode

1st line – glucocorticoid plus mineralocorticoid

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ONGOING
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stable and/or after treatment of acute episode
1st line – 

glucocorticoid plus mineralocorticoid

Oral glucocorticoid and mineralocorticoid replacement therapy is given in physiological doses for life.[2][8]

Generally, short-acting glucocorticoids such as hydrocortisone are preferred by most physicians.[3] If hydrocortisone is not available, alternative glucocorticoids are cortisone and prednisolone.[2][43] The adequacy of glucocorticoid replacement is guided by clinical symptoms. Persistent fatigue, weight loss, and nausea are symptomatic of insufficient dosage.[3] Excessive weight gain or facial plethora is symptomatic of over-replacement.[2]

Mineralocorticoid dose is impacted by mineralocorticoid potency of the glucocorticoid administered and is adjusted based on clinical symptoms (particularly salt craving and postural hypotension) and serum potassium.[2] Excessive mineralocorticoid replacement causes hypertension, hypokalaemia, and oedema.

There may be a physiological increase in glucocorticoid and mineralocorticoid requirement during pregnancy.[2][50] This should be monitored frequently (e.g., every 6 to 8 weeks), and dose adjustments made as appropriate.

Dose varies across guidelines; consult your local guidance for more information.

Primary options

hydrocortisone: consult specialist for guidance on dose

or

cortisone: consult specialist for guidance on dose

or

prednisolone: consult specialist for guidance on dose

-- AND --

fludrocortisone: consult specialist for guidance on dose

Consider – androgen replacement

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ONGOING
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stable and/or after treatment of acute episode
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premenopausal women with decreased libido
Consider – 

androgen replacement

Additional treatment recommended for SOME patients in selected patient group

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.

Routine DHEA (also known as prasterone) replacement is controversial.[51] Some studies have found improved well-being and sexuality in women receiving physiological doses of DHEA.[52][53] However, one study found no benefit associated with DHEA replacement.[54]

Premenopausal women with complaints of decreased libido or sexual well-being despite optimised glucocorticoid and mineralocorticoid replacement may be treated with DHEA replacement.[2]

DHEA should be discontinued periodically to assess these symptoms.

Side effects include signs and symptoms related to hyper-androgenism, including acne, hirsutism, increased sweat odour, scalp itching, acne, and hair loss.[52][54]

Primary options

dehydroepiandrosterone: consult specialist for guidance on dose

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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