Pt is a 55 y/o F w/ hx of SLE who presents to the ED for hypotension. Pt is altered and not providing any information. Pt arrives with family who notes she has had progressively worsening mental status and abdominal pain w/ vomiting non bilious material x 3 days. Not compliant with medications during this time 2/2 vomiting. Unclear what medications she takes.

HR 119, BP 71/48, Temp 94F, RR 20, Acc Check 51

Physical Exam:
GCS 11, arousable to gentle stimuli 

Extremities warm w/o pitting edema

Abdomen soft and nontender

Lungs CTA b/l

Heart rate tachycardia and regular

POCUS unremarkable

Pertinent Labs:

CBC: WBC 15k w/ increased lymphocytosis

BMP: Na 128, K 5.8, AG 18, Cr 2.1

Differential diagnosis: Distributive shock 2/2 sepsis w/ unclear source vs adrenal insufficiency, hypovolemia, myxedema coma etc.

Physiology

• Primary adrenal insufficiency (Addisons) – direct loss of glucocorticoids (cortisol) and mineralocorticoid (aldosterone)
• Secondary adrenal insufficiency – loss of ACTH / CRH
  • More common secondary to chronic steroid therapy – leads to loss of glucocorticoids
  • Aldosterone is largely unaffected because its regulated by RAAS so typical electrolyte abnormalities are not as common / pronounced and hypotension is less severe

When to consider

• Shock refractory to fluid resuscitation / vasopressors
• Typical lab findings: HypoNa, HyperK, HypoGlycemia, HypoThermia, HypoTension, AMS
• Azotemia
• If leukocytosis -> likely eosinophilia w/ lymphocytosis > neutrocytosis
• Fever of unexplained origin

High Risk Groups

• Included in the ddx for shock refractory to pressors / volume resuscitation

• Pts on long term steroids
• Autoimmune disorders
• HIV and TB
• Head trauma, tumors and pituitary apoplexy (sheehan syndrome)
• Bacterial meningitis
• Neonates w/ congenital adrenal hyperplasia
• Infiltrative disorders
• Chronic opioid use and withdraw can suppress the HPA axis as well

Diagnosis

• In the ED, its a clinical diagnosis. Suspect? = Treat!
• Cortisol < 20 ug/dl is highly suggestive however not definitive
• Confirm w/ cosyntropin test (to be done on floor/ICU)

Management

• Hydrocortisone 100mg IV then 50mg q6: 1st line as it has glucocorticoid and mineralocorticoid effects – however it will affect the cosyntropin test. Can consider adding fludrocortisone for added mineralocorticoid benefits but not typically recommended.
  • If affecting the cosyntropin test is a large concern – consider starting dexamethasone 6mg IV (glucocorticoid). Must rx fludrocortisone as well (mineralocorticoid)
• Low threshold for empiric levothyroxine as well
• If pt truly has adrenal crisis – pt should have some clinical improvement within a few hours after receiving the above steroids

Prevention

• Patients on chronic steroids who present w/ acute illness – take at home steroid dose and triple – this is their new maintenance dose for the next 1-3 days or until their acute presentation improves.

References: