Adrenal Crisis (Acute Adrenal Insufficiency)

What happens in an adrenal crisis

The adrenal glands sit on top of the kidneys and make cortisol, which keeps blood pressure and blood sugar up and helps the body respond to stress. Normally, cortisol output rises several-fold during illness or injury.

In adrenal insufficiency, the body cannot make enough cortisol. Day to day, replacement tablets cover the need. But during stress, the usual dose is not enough, and if tablets are vomited up or missed, cortisol falls rapidly. Blood vessels lose their tone, blood pressure collapses, and blood sugar and sodium fall. Without treatment, this leads to shock and death.

Symptoms

Early symptoms can look like a stomach bug or flu:

• severe tiredness and weakness
• nausea, vomiting and loss of appetite
• abdominal, back or leg pain
• dizziness or feeling faint on standing

Then signs of crisis develop:

• confusion, drowsiness or agitation
• very low blood pressure, rapid pulse, cold clammy skin
• fever (often from the trigger infection)
• low blood sugar, especially in children
• collapse and loss of consciousness

Darkened skin, salt craving and weight loss in the weeks beforehand suggest undiagnosed Addison's disease.

Causes and risk factors

Underlying conditions

• Primary adrenal insufficiency (Addison's disease): autoimmune destruction of the adrenal glands, or rarely TB, infections, cancer or bleeding into the glands.
• Secondary adrenal insufficiency: hypopituitarism from pituitary tumors, surgery or radiation.
• Congenital adrenal hyperplasia: an inherited enzyme deficiency.
• Steroid-induced (tertiary) adrenal insufficiency: long-term prednisone, dexamethasone, high-dose inhaled steroids or steroid injections suppress the body's own production. Stopping them suddenly can trigger a crisis.
• Adrenal hemorrhage: bleeding into both adrenal glands, for example with severe infection (meningococcal sepsis) or blood thinners.

Triggers

• infections, especially gastroenteritis with vomiting and diarrhea
• injury, burns, surgery, dental procedures or childbirth
• missed doses, or suddenly stopping long-term steroids
• severe emotional or physical stress, intense exercise in heat
• starting some medicines that speed up cortisol breakdown (such as rifampin) or thyroid hormone in someone with undiagnosed adrenal insufficiency

Emergency treatment

What patients and families do

If someone with adrenal insufficiency is vomiting, collapsing or very unwell:

1. Give the emergency hydrocortisone injection at the dose prescribed for that person (children's doses are lower) into the outer thigh muscle. It can be given through clothing.
2. Call 911 and tell them the person has adrenal insufficiency and has had an injection.
3. Show the steroid emergency card or medical ID.
4. Lay the person down with legs raised; if they are unresponsive but breathing, use the recovery position.

The injection cannot cause harm in this situation. If in doubt, inject.

In hospital

• High-dose IV or IM hydrocortisone immediately, continued over the next 24 hours by infusion or repeated doses, then tapered as the person recovers
• IV saline to restore blood pressure, with glucose if blood sugar is low
• tests: sodium, potassium, glucose, kidney function, cortisol and ACTH (drawn before steroids if possible, but treatment must never be delayed for tests)
• treatment of the trigger, such as antibiotics for infection

Most people improve dramatically within hours.

Preventing adrenal crisis

Sick day rules

• Fever or illness needing bed rest: double the usual daily hydrocortisone (or other glucocorticoid) dose until recovered, typically for 2 to 3 days.
• Fever above 38 °C (100.4 °F) or significant illness: some guidelines advise tripling the dose. Follow your own endocrinologist's plan.
• Vomiting or unable to take tablets: use the emergency injection and seek urgent care.
• Surgery and procedures: extra steroid cover is needed; tell every clinician that you take steroids.

Be prepared

• Carry an emergency injection kit and know how to use it; teach family members.
• Wear medical ID and carry a steroid emergency card.
• Never stop long-term steroids suddenly; they must be tapered under medical advice.
• Keep spare tablets when traveling.

Outlook

With prompt treatment, most people recover fully from an adrenal crisis. Crises still occur in about 6 to 8 per 100 patients with adrenal insufficiency each year, and they remain a cause of preventable death. Education on sick day rules and emergency injection use is the most effective protection.

Frequently asked questions

What are the first signs of an adrenal crisis?

Early signs include extreme tiredness, weakness, nausea, vomiting, abdominal or back pain, and dizziness on standing. These can progress quickly to confusion, very low blood pressure and collapse. Anyone with adrenal insufficiency who is vomiting needs urgent treatment.

What triggers an adrenal crisis?

Common triggers are infections (especially stomach bugs with vomiting and diarrhea), injury, surgery, missed steroid doses, and suddenly stopping long-term steroids. Any physical stress raises cortisol needs that someone with adrenal insufficiency cannot meet without extra medicine.

How is an adrenal crisis treated?

An immediate high-dose hydrocortisone injection, followed by IV fluids, more hydrocortisone, glucose if needed, and treatment of the trigger in hospital. People at risk carry an emergency injection kit so it can be given before reaching hospital.

Can long-term prednisone cause an adrenal crisis?

Yes. Taking steroids such as prednisone for more than a few weeks suppresses the body's own cortisol production. Stopping suddenly, or facing serious illness or surgery without extra steroid cover, can trigger an adrenal crisis. Steroids should be tapered under medical guidance.

What are steroid sick day rules?

Sick day rules tell people on steroid replacement to double (sometimes triple) their dose when ill with fever or needing bed rest, and to use their emergency injection and seek urgent care if vomiting or unable to take tablets. Your endocrinologist will give you a personal plan.