Adrenal crisis

Background

  • Adrenal insufficiency occurs when the adrenal glands fail to supply the physiologic demands of the body for glucocorticoids (cortisol) and/or mineralocorticoids (aldosterone).
  • Divided into primary adrenal insufficiency or secondary adrenal insufficiency
  • Adrenal crisis is the acute, life-threatening presentation of adrenal insufficiency[1]
    • 8-47% of patients with primary adrenal insufficiency will have at least one adrenal crisis in their lives
    • Consider in any patient with unexplained hypotension (especially in those with HIV or taking exogenous steroids)
    • Generally caused by mineralocorticoid deficiency, not glucocorticoid deficiency
      • This is the reason crises occur much more frequently with primary adrenal insufficiency

Causes (Adrenal Insufficiency)

  • Primary adrenal insufficiency (decreased cortisol and aldosterone)
    • Autoimmune (70%)
    • Adrenal hemorrhage
      • Coagulation disorders
      • Sepsis (Waterhouse-Friderichsen syndrome)
    • Meds
    • Infection (HIV, TB)
      • TB is most common worldwide cause primary adrenal insuffiency
    • Sarcoidosis/amyloidosis
    • Metastases
    • CAH
  • Secondary adrenal insufficiency (decreased ACTH → decreased cortisol only)
    • Withdrawal of steroid therapy
    • Pituitary disease
    • Head trauma
    • Postpartum pituitary necrosis
    • Infiltrative disorders of pituitary or hypothalamus

Precipitants

Clinical Features

  • Secondary Adrenal Insufficiency
    • Similar to primary adrenal insufficiency
    • No hyperpigmentation
    • No hyperkalemia
    • Hypotension less common

Differential Diagnosis

Shock

Evaluation

  • Imaging
    • Consider CXR to identify infectious triggers

Management

Begin treatment immediately in any suspected case (prognosis related to rapidity of treatment)

  • Treat underlying cause, if known
  • IVF - D5NS 2-3L (corrects fluid deficit and hypoglycemia)
  • Steroids
    • Hydrocortisone - 2mg/kg up to 100mg IV bolus
      • Drug of choice if K+>6 (provides glucocorticoid and mineralocorticoid effects)
    • Dexamethasone - 4mg IV bolus
      • Consider in hemodynamically stable patients if ACTH stimulation test will be performed (will not interfere with the test)
      • Along with methylprednisolone, dexamethasone has negligible mineralocorticoid effect, so choose hydrocortisone in[6]:
    • Comparable steroid dosages
  • Vasopressors
    • Administer after steroid therapy in patients unresponsive to fluid resuscitation

Stress-Dose Steroids in Illness

To aid in mounting stress response in those with adrenal insufficiency lacking endogenous cortisol

Illness Type Steroid Administration
Minor, with fever < 38°C Double dose of chronic maintenance steroids
Severe, with fever > 38°C Triple dose of chronic maintenance steroids
Vomiting, listless, or hypotensive Hydrocortisone at 1-2mg/kg (as above in adrenal crisis)

Disposition

  • Admission
    • Admit all patients with acute adrenal insufficiency, especially if new diagnosis for the patient
    • Patients with adrenal crisis should receive ICU admission
  • Discharge
    • Consult endocrinology if discharge considered for mild cases w/ normal lab values

See Also

External Links

References

  1. Hahner S, Loeffler M, Bleicken B, et al. Epidemiology of adrenal crisis in chronic adrenal insufficiency: the need for new prevention strategies. Eur J Endocrinol. 2010;162(3):597-602. doi:10.1530/EJE-09-0884
  2. Rao RH, Vagnucci AH, Amico JA. Bilateral massive adrenal hemorrhage: early recognition and treatment. Ann Intern Med. 1989;110(3):227-235. doi:10.7326/0003-4819-110-3-227
  3. Izumi Y et al. Renal tubular acidosis complicated with hyponatremia due to cortisol insufficiency. Oxf Med Case Reports. 2015 Nov; 2015(11): 360–363.
  4. Bancos I, Hahner S, Tomlinson J, Arlt W. Diagnosis and management of adrenal insufficiency. Lancet Diabetes Endocrinol. 2015;3(3):216-226. doi:10.1016/S2213-8587(14)70142-1
  5. Nutman TB. Evaluation and differential diagnosis of marked, persistent eosinophilia. Immunol Allergy Clin North Am. 2007 Aug; 27(3): 529–549.
  6. Wilson TA et al. Adrenal Hypoplasia Medication. eMedicine. Feb 11, 2013. http://emedicine.medscape.com/article/918967-medication.