Endocrinology · Adrenal Insufficiency
The facts most likely to be tested
Adrenal crisis presents with refractory hypotension that is unresponsive to intravenous fluids and vasopressors.
The most common precipitating factor is abrupt withdrawal of long-term glucocorticoid therapy.
Laboratory findings classically demonstrate hyponatremia, hyperkalemia, and hypoglycemia due to mineralocorticoid and glucocorticoid deficiency.
The immediate first-line treatment is intravenous hydrocortisone and aggressive isotonic saline resuscitation.
Cosyntropin stimulation testing is the diagnostic gold standard, but treatment must never be delayed for testing in a hemodynamically unstable patient.
Primary adrenal insufficiency is distinguished from secondary causes by the presence of hyperpigmentation due to elevated ACTH and MSH levels.
Fludrocortisone is added to the regimen only after the patient is stable and able to tolerate oral medications to address mineralocorticoid deficiency.
Vignette unlocked
A 45-year-old female with a history of rheumatoid arthritis is brought to the emergency department with severe nausea, vomiting, and confusion. She recently stopped taking her daily prednisone taper against medical advice. On physical exam, she is lethargic with a blood pressure of 80/50 mmHg and a heart rate of 120 bpm. Labs reveal a sodium of 128 mEq/L, potassium of 5.8 mEq/L, and glucose of 55 mg/dL. Despite two liters of normal saline, her blood pressure remains 82/52 mmHg.
What is the most appropriate next step in management?
Intravenous hydrocortisone
The patient is in adrenal crisis due to steroid withdrawal, characterized by refractory hypotension and electrolyte abnormalities; immediate replacement of glucocorticoids is life-saving.
Full handout
High yield triage
Etiology / Epidemiology
Occurs in patients with primary adrenal insufficiency (Addison's disease) or those on chronic exogenous steroids who experience acute stress.
Clinical Manifestations
Presents as refractory hypotension unresponsive to fluids/pressors, often with hyperpigmentation in primary cases.
Diagnosis
Diagnosis is confirmed via Cosyntropin stimulation test; do not delay treatment for testing.
Treatment
Immediate IV hydrocortisone is the gold standard; never delay treatment for diagnostic testing.
Prognosis
High mortality if untreated; 100% survival with prompt recognition and aggressive fluid resuscitation.
Full handout
Epidemiology & Etiology
Most commonly triggered by abrupt withdrawal of chronic glucocorticoids or acute physiologic stress (infection, trauma, surgery) in patients with undiagnosed Addison's disease. Patients with autoimmune adrenalitis are at highest risk. Always consider in patients with unexplained shock.
Pertinent Anatomy
The adrenal cortex produces cortisol (zona fasciculata) and aldosterone (zona glomerulosa). Loss of these hormones leads to the classic triad of hypotension, hyponatremia, and hyperkalemia.
Pathophysiology
Acute deficiency of cortisol results in loss of vascular tone and sensitivity to catecholamines. Concomitant aldosterone deficiency causes profound salt wasting and volume depletion. This creates a state of distributive shock that is resistant to standard vasopressors.
Clinical Manifestations
Patients present with refractory hypotension, abdominal pain, and altered mental status. Look for hyperpigmentation (due to elevated ACTH) in primary insufficiency. Red flags include hypoglycemia and hyperkalemia which can lead to fatal arrhythmias.
Diagnosis
The Cosyntropin stimulation test is the gold standard; a serum cortisol level < 18 mcg/dL 30-60 minutes post-injection confirms the diagnosis. Obtain a random serum cortisol and ACTH level prior to treatment if possible, but do not delay life-saving therapy.
Treatment
Initiate IV hydrocortisone (100mg bolus) immediately. Provide aggressive IV isotonic saline resuscitation to correct volume depletion. Avoid etomidate for intubation as it inhibits adrenal steroidogenesis. Transition to oral maintenance once stable.
Prognosis
Prognosis is excellent with early glucocorticoid replacement. Monitor for hypoglycemia and electrolyte imbalances during the acute phase. Long-term management requires patient education on stress-dose steroids for future illness.
Differential Diagnosis
Septic shock: usually associated with fever and source of infection
Hypovolemic shock: lacks the characteristic hyperkalemia/hyponatremia
Myxedema coma: presents with bradycardia and hypothermia
Diabetic ketoacidosis: presents with hyperglycemia rather than hypoglycemia
Pituitary apoplexy: sudden headache and visual field deficits