Presentation

Classic presentation

• Patients with adrenal insufficiency usually present with nonspecific symptoms including weakness/fatigue, weight loss, anorexia, abdominal pain, fever, and depression.
  
• The classic finding in adrenal crisis is refractory hypotension.
  
• These patients can frequently appear to be in septic shock, and they may in fact be septic, but their body is not able to respond appropriately because of a lack of cortisol.
  
• A thorough history including steroid use/drug use, surgeries, history of HIV, and precipitating symptoms should be elicited.

Critical presentation

• Consider adrenal crisis in the patient who is persistently hypotensive despite multiple liters of fluid boluses and high doses of multiple vasopressors.
  
• Acute adrenal insufficiency can appear to be a severe gastroenteritis with fever, vomiting, and dehydration, but this can quickly progress to vascular collapse and death.

Diagnosis and evaluation

• Vital signs are the main identifier of illness since the symptoms are nonspecific.
  
  
  
  • Refractory hypotension is the hallmark of adrenal crisis as discussed above.
    
  • The lack of reflexive tachycardia in a hypotensive state should increase concern for adrenal crisis since cortisol deficiency blunts the physiological vasoconstriction and catecholamine synthesis. However, many patients are on beta-blockers, calcium channel blockers or other antiarrhythmic drugs that can cause this discrepancy.
    
  • Consider adrenal crisis or neurogenic shock in a hypotensive-normocardic patient not taking heart-rate-modifying medications.
  
  
• Physical examination is frequently unimpressive, but a thorough examination can help determine the underlying cause.
  
  
  
  • First assess for signs of infection, which can precipitate a crisis:
    
    
    
    • Focal lung findings: crackles or rhonchi or wheezes
      
    • Altered mental status
      
    • Abdominal tenderness
      
    • Rashes
      
    • Joint swelling and erythema.
    
    
  • Dermatological examination can be especially helpful if adrenal crisis is suspected:
    
    
    
    • Hyperpigmentation of sun-exposed areas, axilla, palmar creases, and mucous membranes (Addison disease). The increased ACTH secreted by the pituitary stimulates melanin production.
      
    • Petechial rash (meningococcemia).
      
    • Vitiligo (white patches of amelanotic skin) can be seen in polyglandular autoimmune (PGA) syndrome type I.
  
  
• Diagnostic tests
  
  
  
  • Laboratory tests:
    
    
    
    • Hyponatremia is seen in primary adrenal insufficiency because of the aldosterone deficiency. Additionally, the lack of cortisol leads to increased antidiuretic hormone (ADH) secretion, increased free water absorption, and worsening hyponatremia.
      
    • The lack of aldosterone also causes hyperkalemia, with an accompanying mild hyperchloremic metabolic acidosis.
      
    • Hypoglycemia can be seen in both primary and secondary adrenal insufficiency because of a lack of cortisol and decreased appetite. Additionally, in secondary adrenal insufficiency, growth hormone and ACTH deficiency contribute further to hypoglycemia.
    
    
  • Serum cortisol level:
    
    
    
    • Should be elevated with acute illness because of physiological stress on the body.
      
    • A level <15–18 micrograms/mL is diagnostic of adrenal crisis in an acutely ill patient. A level >33 micrograms/mL excludes the diagnosis.
      
    • If the level is indeterminate, an ACTH (cosyntropin) stimulation test with less than a 9 micrograms/mL increase in serum cortisol is also diagnostic. Dexamethasone does not alter the cortisol assay and can be used instead of hydrocortisone if treatment is emergent and a stimulation test will be performed later.
    
    
  • Keep in mind that laboratories currently measure total cortisol only and the majority of cortisol is protein bound. As such, cortisol values might not reflect the true active cortisol level in the patient.

Critical management

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