Hepatic Failure/GI/Endocrine Emergencies

Adrenal crisis

Clinical presentation

Because there is no universally accepted evidence-based definition of adrenal crisis, cases

are identified based on a pragmatic definition and clinical assessment. In adults, adrenal

crisis is defined as an acute deterioration in health status associated with absolute or relative

hypotension (SBP less than 100 mm Hg or 20 mm Hg or more below baseline) that improves

within 1–2 hours after parenteral glucocorticoid administration. Adrenal crisis is a rare cause

of vasodilatory shock.

ii.

Patients with acute adrenal insufficiency typically present with severe hypotension, altered

mental status, acute abdominal pain, vomiting, and fever. As such, patients often receive a

misdiagnosis of an acute abdomen. Accompanying symptoms may include fatigue, lethargy,

confusion, weakness, and pain in the muscles or joints. Patients with primary adrenal

insufficiency may also have salt craving (because of mineralocorticoid deficiency secondary

to a lack of aldosterone). Characteristic skin hyperpigmentation from primary adrenal

insufficiency may also be present.

iii.

Laboratory findings will include low serum cortisol (i.e., cortisol level below 18 mcg/dL in an

acutely stressed patient is suggestive of adrenal insufficiency); hyponatremia, hyperkalemia,

hypoglycemia, and normocytic anemia may also be present.

Causes

Adrenal crisis may result from primary adrenal failure or secondary adrenal disease.

ii.

Acute-onset primary adrenal insufficiency is most often caused by adrenal hemorrhage or

infarction. Common causes of secondary adrenal crisis include pituitary apoplexy, pituitary or

hypothalamic surgery, or traumatic brain injury.

iii.

Patients on chronic corticosteroid therapy who experience acute stress (e.g., infection or surgery)

will be unable to increase their cortisol appropriately because of suppression of corticotropin-

releasing hormone and corticotropin.

iv.

Bacterial infections commonly precipitate adrenal crisis in adults. In children, viral infections

are more common precipitating events.

Medications may contribute to inadequate serum cortisol concentrations, including etomidate

and ketoconazole. Adrenal crisis has also been reported after vaccinations and zoledronic acid

infusions. Case series describe adrenal insufficiency following immune checkpoint inhibitor

therapy.

vi.

Abrupt decrease in dose or cessation of corticosteroids in patients receiving moderate to high

doses (greater than or equal to prednisone 7.5 mg daily or equivalent) for 3 weeks or more may

precipitate adrenal crisis.

Management

Treatment should be initiated as soon as diagnosis is suspected and does not need to be delayed

for the results of diagnostic tests.

ii.

Patients with acute adrenal crisis should receive intravenous fluids and vasopressors if shock is

present. After initial bolus, dextrose may be added for treatment of hypoglycemia.

iii.

Glucocorticoids should be administered quickly. Hydrocortisone is preferred because of its

protein binding, tissue distribution, and balanced glucocorticoid and mineralocorticoid effects;

it is typically given as a 100 mg intravenous initial bolus followed by 50 mg intravenously

every 6 hours. Alternatives include methylprednisolone 20 mg intravenously twice daily or

dexamethasone 4–6 mg intravenously daily. Doses can be tapered based on patient’s clinical

status.