Adrenal Crisis from Corticosteroid Withdrawal
Diagnosis and Presentation
- The American College of Physicians recommends that adrenal crisis be considered in patients with a history of chronic corticosteroid use who present with periumbilical abdominal pain, nausea, and electrolyte abnormalities 1, 2, 3
- Hyponatremia is present in 90% of newly diagnosed adrenal insufficiency cases, and hypokalemia rather than hyperkalemia occurs when vomiting is present, as aldosterone deficiency is masked by gastrointestinal losses 1, 2
- The absence of hyperkalemia is misleading, as it is only present in ~50% of adrenal insufficiency cases, and vomiting causes hypokalemia that masks the expected hyperkalemia 1, 2, 4
Diagnostic Workup
- The Endocrine Society recommends drawing morning cortisol and ACTH before any steroid administration, and performing a cosyntropin stimulation test to confirm secondary adrenal insufficiency 1, 4
- Cortisol <250 nmol/L with low/normal ACTH confirms secondary adrenal insufficiency, and cortisol <400 nmol/L with clinical instability warrants empiric treatment 1, 4
- Peak cortisol <500 nmol/L is diagnostic of adrenal insufficiency, and this test is the gold standard when initial cortisol is indeterminate 1, 4
Management
- The American College of Emergency Physicians recommends giving 100 mg IV hydrocortisone immediately if the patient is clinically unstable, and infusing 1 L of 0.9% normal saline over 1 hour 2, 3, 4
- If the patient is stable enough for testing, drawing morning cortisol and ACTH immediately, and performing a cosyntropin stimulation test is recommended 1, 4
- If the test confirms adrenal insufficiency, initiating stress-dose steroids and tapering to maintenance dosing over 2-3 days once stable is recommended 3, 4
Long-Term Considerations
- The Endocrine Society recommends lifelong glucocorticoid replacement therapy if the HPA axis does not recover, and stress-dose education and medical alert identification are mandatory 4
- Endocrine follow-up to assess for HPA axis recovery, which may take weeks to months, and screening for other autoimmune conditions if primary adrenal insufficiency is found instead of secondary are recommended 1, 4