An adrenal crisis is a potentially life-threatening condition of mineralocorticoid deficiency in the presence of chronic adrenal insufficiency. Patients with adrenal insufficiency are at risk for adrenal crisis with acute illness or abrupt cessation of steroid medications. The most common precipitating events are gastrointestinal infection, fever, acute stress, and untreated or undertreated adrenal and pituitary disorders.
Adrenal crisis is primarily characterized by shock and hypotension. It may also present with sudden abdominal pain, back or leg pain, nausea, vomiting, diarrhea, a change in mental status or delirium, Addison disease, hyperkalemia, and/or hyponatremia. Other symptoms may include anorexia, weakness, lethargy, hypoglycemia, and coma.
Adrenal crisis accounts for excess mortality in patients with adrenal insufficiency. The incidence of adrenal crisis in patients with adrenal insufficiency on hormone replacement therapy is 5-10 crises per 100 patient years. Patients can decompensate rapidly, with crises evolving within just a few hours after the first symptoms present, making recognition and immediate treatment critical.
Related topics: Addison disease, Secondary adrenal insufficiency
Potentially life-threatening emergency
Adrenal crisis
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Synopsis
Codes
ICD10CM:
E27.2 – Addisonian crisis
SNOMEDCT:
24867002 – Severe adrenal insufficiency
E27.2 – Addisonian crisis
SNOMEDCT:
24867002 – Severe adrenal insufficiency
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Addison disease – autoimmune disorder, associated with elevated adrenocorticotropic hormone (ACTH)
- Shock – septic, cardiogenic, anaphylaxis, etc
- Adrenal hemorrhage
- Waterhouse-Friderichsen syndrome – associated with bacterial infection, especially Neisseria meningitidis
- Opportunistic infections – disseminated fungal infections can cause primary adrenal insufficiency, especially in the setting of HIV infection
- Sarcoidosis – caused by granuloma in adrenal glands
- Tuberculosis – most common cause of primary adrenal insufficiency worldwide
- Sheehan syndrome – hypopituitarism resulting after severe postpartum hemorrhage
- Malignancy (particularly metastasis, primary adrenal, renal, pituitary)
- Congenital adrenal hyperplasia – often associated with salt wasting and virilization
- Trauma (see, eg, traumatic brain injury)
- Genetic conditions – mutations in genes related to pituitary development (including HESX homeobox 1 gene, orthodenticle homeobox 2 gene, LIM homeobox 4 gene, SRY gene, congenital proopiomelanocortin deficiency, and Prader-Willi syndrome)
- Adrenoleukodystrophy – X-linked peroxisomal disease
- Hemochromatosis
- Amyloidosis (AA amyloidosis, AL amyloidosis)
- Pituitary insufficiency
- Iatrogenic – medications including chlorpromazine, imipramine, etomidate, ketoconazole, fluconazole, suramin, phenytoin, barbiturates, mifepristone, rifampin, and opiates
- Chronic corticosteroid use – common cause of tertiary adrenal insufficiency, suppresses hypothalamic-pituitary-adrenal axis; highest risk for adrenal crisis with acute illness or abrupt cessation of high-dose corticosteroids
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Management Pearls
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Therapy
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Drug Reaction Data
Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.
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References
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Last Reviewed:05/06/2019
Last Updated:05/19/2019
Last Updated:05/19/2019