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Adrenal Crisis / Emergencies – Causes, Symptoms, Treatment

November 9, 2021
Adrenal crisis (also known as Addisonian crisis and acute adrenal insufficiency) is a medical emergency and potentially life-threatening situation requiring immediate emergency treatment. It is a constellation of symptoms that indicate severe adrenal insufficiency caused by insufficient levels of the hormone cortisol. This may be the result of either previously undiagnosed or untreated Addison’s disease, a disease process suddenly affecting adrenal function (such as bleeding from the adrenal glands in Waterhouse-Friderichsen syndrome), suddenly stopping the intake of glucocorticoids or an intercurrent problem (e.g. infection, trauma, in fact, any form of physical or mental stress) in someone known to have Addison’s disease, congenital adrenal hyperplasia (CAH), or another form of primary adrenal insufficiency.

Adrenal Emergencies

Hormones: aldosterone, cortisol, androgens, catecholamines

Causes of Adrenal insufficiency

Adrenal crisis is caused by a deficiency of cortisol resulting from Addison’s disease, congenital adrenal hyperplasia (CAH), corticosteroid biosynthetic enzyme defects or pituitary disorders (such as Sheehan’s syndrome, pituitary adenoma, hypopituitarism (inactive or underactive pituitary) causing failure to activate the adrenal glands.

Primary

  • Causes
    • Autoimmune (associated with other endocrinopathies, PTH, DM)
    • Infection (TB, viral, meningococcemia)
    • Infiltration (sarcoidosis, amyloidosis)
    • Hemorrhage (trauma, anti-coagulation)
    • Malignancy (primary, metastatic)
  • Secondary
    • Causes
      • Iatrogenic (>5mg prednisone/day for > 2 weeks)
      • Pituitary/sellar tumors
      • Hemorrhage (Sheehan)
      • Cranial radiation

Signs and symptoms

Characteristic symptoms are

  • Sudden penetrating pain in the legs, lower back or abdomen
  • Confusion, psychosis, slurred speech
  • Severe lethargy
  • Convulsions
  • Fever
  • Hyperkalemia (elevated potassium level in the blood)
  • Hypercalcemia (elevated calcium level in the blood): the cause of hypercalcemia is a combination of increased calcium input into the extracellular space and reduced calcium removal by the kidney, this last caused by decreased glomerular filtration and increased tubular calcium reabsorption. Both renal factors are secondary to volume depletion and, in fact, improve rapidly during rehydration with saline infusion.[rx]
  • Hypoglycemia (reduced level of blood glucose)
  • Hyponatremia (low sodium level in the blood)
  • Hypotension (low blood pressure)
  • Hypothyroid (low T4 level)
  • Severe vomiting and diarrhea, resulting in dehydration
  • Syncope (loss of consciousness and ability to stand)
  • AIMS
  • Hypotension (refractory)
  • GI: anorexia, nausea/vomiting, diarrhea
  • Hyperpigmentation

Diagnosis

Labs

  • Hyponatremia
  • Hyperkalemia
  • Hypercalcemia
  • Mild metabolic acidosis
  • Hypoglycemia
  • Various investigations aid the diagnosis.
    • ACTH (cosyntropin) stimulation test
    • Cortisol level (to assess the level of glucocorticoids)
    • Fasting blood sugar
    • Serum potassium
  • Signs/Symptoms
    • RAAS function maintained, hypotension rare
    • Features of pituitary/hypothalamic dysfunction: menstrual disturbances, headache, vision changes, galactorrhea, acromegaly
  • Adrenal Crisis
    • Precipitated by physiologic stressor: sepsis, MI, trauma, surgery
    • Diagnosis
      • AM cortisol <3
      • ACTH stimulation peak cortisol <15
      • ACTH level
    • Management
      • Glucose management
      • Fluid resuscitation
      • Dexamethasone 10mg IV
      • Identify and treat precipitant

Treatment

Acute adrenal insufficiency is a medical emergency and needs to be treated with injectable hydrocortisone and fluid support.

Prevention

An adrenal crisis is triggered by physiological stress (such as trauma). Activities that have an elevated risk of trauma are best avoided. Treatment must be given within two hours of trauma and consequently, it is advisable to carry injectable hydrocortisone in remote areas.

References

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