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Addisonian Crisis: Causes, Symptoms & Treatment

An Addisonian crisis is a life-threatening emergency caused by a severe lack of cortisol and aldosterone. Immediate medical treatment is essential.

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Things worth knowing about "Addison Crisis"

An Addisonian crisis is a life-threatening emergency caused by a severe lack of cortisol and aldosterone. Immediate medical treatment is essential.

What is an Addisonian Crisis?

An Addisonian crisis (also known as an adrenal crisis or acute adrenal insufficiency) is a sudden, life-threatening medical emergency caused by a severe deficiency of the vital hormones produced by the adrenal cortex -- primarily cortisol and aldosterone. Without immediate treatment, an Addisonian crisis can be fatal within hours.

The condition is named after the British physician Thomas Addison, who first described chronic adrenal insufficiency (known as Addison's disease) in the 19th century. The crisis represents the acute, decompensated form of this underlying condition.

Causes

An Addisonian crisis can be triggered by several factors:

Known Addison's disease: Patients with a diagnosed adrenal insufficiency who miss their medication or fail to increase their dose during physical stress such as infection, surgery, or injury.
First presentation: An undiagnosed adrenal insufficiency that becomes apparent for the first time during a period of physical stress.
Abrupt discontinuation of corticosteroid therapy: Patients who have been taking corticosteroids long-term and suddenly stop.
Bilateral adrenal hemorrhage (Waterhouse-Friderichsen syndrome): Bleeding into both adrenal glands, often associated with severe sepsis.
Surgical removal of the adrenal glands without adequate hormone replacement therapy.
Pituitary failure: Failure of the pituitary gland, which controls the adrenal glands (secondary adrenal insufficiency).

Symptoms

The symptoms of an Addisonian crisis develop rapidly and can be life-threatening:

Severe drop in blood pressure (hypotension) leading to circulatory shock
Extreme weakness and fatigue
Nausea, vomiting, and diarrhea
Severe abdominal pain
Confusion, loss of consciousness, or disorientation
High fever
Hypoglycemia (low blood sugar)
Hyponatremia (low sodium) and hyperkalemia (high potassium)
Tachycardia (rapid heart rate)

Diagnosis

The diagnosis of an Addisonian crisis is primarily a clinical emergency diagnosis. Treatment must not be delayed while waiting for laboratory results. The following tests are performed:

Blood tests: Measurement of cortisol, sodium, potassium, blood glucose, complete blood count, and kidney function markers
ACTH stimulation test: To confirm adrenal insufficiency (performed after the crisis has resolved)
Blood pressure monitoring and ECG
Imaging (CT or MRI of the adrenal glands) if the cause is unclear

Treatment

Treatment of an Addisonian crisis is a medical emergency and must be initiated immediately:

Immediate cortisol administration: Intravenous or intramuscular injection of hydrocortisone (e.g., 100 mg IV bolus, followed by continuous infusion or every 6 hours)
Intravenous fluid therapy: Normal saline (0.9% NaCl) to stabilize blood pressure and correct salt depletion
Glucose infusion: To correct low blood sugar levels
Electrolyte correction: Normalization of sodium and potassium levels
Treatment of the underlying trigger: e.g., antibiotics for infections
Monitoring in an intensive care unit until stabilization

Emergency Card and Emergency Kit

Patients with known Addison's disease should always carry an emergency medical card and keep an emergency hydrocortisone injection kit for self-administration. In situations where immediate medical help is unavailable, self-injection can be lifesaving. Family members and caregivers should be trained in its use.

Prevention

Preventing an Addisonian crisis in at-risk patients includes:

Taking prescribed hormone replacement therapy regularly (e.g., hydrocortisone, fludrocortisone)
Dose adjustment during stress (e.g., infection, surgery): doubling or tripling the cortisol dose as directed by a physician (the so-called sick day rules)
Carrying an emergency medical identification card
Education of the patient and their caregivers
Regular follow-up with an endocrinologist

References

Bornstein SR et al. - Diagnosis and Treatment of Primary Adrenal Insufficiency: An Endocrine Society Clinical Practice Guideline. Journal of Clinical Endocrinology & Metabolism, 2016.
Husebye ES, Allolio B, Arlt W et al. - Consensus statement on the diagnosis, treatment and follow-up of patients with primary adrenal insufficiency. Journal of Internal Medicine, 2014.
Rushworth RL, Torpy DJ, Falhammar H - Adrenal Crisis. New England Journal of Medicine, 2019.

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