Adrenal crisis

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Background

Posterior view of the adrenal glands (colored) in relation to the viscera and large vessels of the abdomen (thoracic vertebra removed).
Types of adrenal insufficiency (primary, secondary, and tertiary) compared to normal physiology. In its normal state, the hypothalamus secretes corticotropin-releasing hormone (CRH) to induce the pituitary gland to secrete adrenocorticotropic hormone (ACTH) to induce the adrenal gland to secrete cortisol. Cortisol then exerts negative feedback by inhibiting the secretion of CRH and ACTH in the hypothalamus and pituitary gland, respectively.
  • 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 of 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

Forty-nine year-old with an adrenal crisis. Appearance, showing lack of facial hair, dehydration, Queen Anne’s sign (panel A), pale skin, muscular and weight loss, and loss of body hair (panel B)..
  • Secondary Adrenal Insufficiency
    • Similar to primary adrenal insufficiency
    • No hyperpigmentation
    • No hyperkalemia
    • Hypotension less common

Differential Diagnosis

Adrenal crisis

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.
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