Adrenal crisis
(Redirected from Adrenal Insufficiency)
Background
- Life-threatening emergency resulting from acute cortisol deficiency
- Mortality up to 25% if untreated; rapidly fatal without intervention[1]
- Most common cause: stress event in patient with chronic adrenal insufficiency on glucocorticoid replacement who does NOT increase dose ("stress dosing")
- Can also occur as first presentation of undiagnosed adrenal insufficiency
Causes of Adrenal Insufficiency
- Primary (adrenal gland destruction):
- Autoimmune adrenalitis (Addison disease — most common in developed countries)
- Infections: TB (most common worldwide), CMV, HIV, fungal
- Bilateral adrenal hemorrhage (Waterhouse-Friderichsen syndrome — meningococcemia, anticoagulation, DIC)
- Metastatic disease, bilateral adrenalectomy
- Secondary (pituitary — ACTH deficiency):
- Pituitary tumors, surgery, radiation, Sheehan syndrome
- Tertiary (hypothalamic — MOST COMMON overall):
- Chronic exogenous glucocorticoid use → HPA axis suppression
- Even short courses >2 weeks can suppress HPA axis
- Abrupt discontinuation → adrenal crisis
Precipitants of Crisis
- Infection/sepsis (most common trigger)
- Surgery, trauma, critical illness
- Non-compliance or abrupt withdrawal of chronic steroids
- GI illness with vomiting (unable to take oral steroids)
- Emotional stress, adrenal hemorrhage
Clinical Features
- Refractory hypotension/shock — does NOT respond to IV fluids or vasopressors until cortisol replaced
- Weakness, fatigue, lethargy → obtundation → coma
- Nausea, vomiting, abdominal pain (may mimic acute abdomen)
- Fever or hypothermia
- Hypoglycemia (especially in children; cortisol is counterregulatory)
- Dehydration (cortisol deficiency + aldosterone deficiency in primary AI)
- In chronic primary AI: hyperpigmentation (increased ACTH → MSH), vitiligo, salt craving
Classic Lab Pattern
- Hyponatremia (most common electrolyte abnormality)
- Hyperkalemia (primary AI only — aldosterone deficiency; absent in secondary/tertiary)
- Hypoglycemia
- Eosinophilia (cortisol normally suppresses eosinophils)
- Metabolic acidosis, elevated BUN (dehydration)
Differential Diagnosis
- Sepsis / septic shock (most common misdiagnosis — and most common precipitant)
- Thyroid storm / myxedema coma
- Diabetic ketoacidosis
- Hypovolemic shock
- Acute abdomen (gastroenteritis, pancreatitis)
- Drug withdrawal
- Pheochromocytoma crisis
Evaluation
- Random cortisol level (draw BEFORE giving steroids if possible, but do NOT delay treatment):
- Cortisol <3 mcg/dL: diagnostic of adrenal insufficiency
- Cortisol 3-18 mcg/dL in acutely stressed patient: suspicious (should be elevated in stress)
- Cortisol >18 mcg/dL in acute illness: effectively rules out AI
- ACTH level (distinguish primary vs secondary):
- High ACTH = primary AI; Low/normal ACTH = secondary/tertiary
- BMP: hyponatremia, hyperkalemia, hypoglycemia, metabolic acidosis
- CBC: eosinophilia, lymphocytosis
- Blood glucose (POC immediately)
- TSH (concurrent hypothyroidism in autoimmune polyendocrine syndrome)
- Infectious workup: blood cultures, UA, CXR, lactate (identify precipitant)
- ACTH stimulation test (cosyntropin test): NOT needed acutely — do NOT delay treatment for this test
Management
Immediate
- Hydrocortisone 100 mg IV bolus — give immediately if suspected (even before lab confirmation)
- Then hydrocortisone 50 mg IV q6-8h (or continuous infusion 200 mg/24h)
- If hydrocortisone unavailable: dexamethasone 4 mg IV (does not interfere with subsequent cortisol testing)
- IV fluids: aggressive NS resuscitation (patients are often 2-3L volume depleted)
- D5NS if hypoglycemic — correct hypoglycemia with D50W 25-50 mL IV
- Vasopressors if refractory hypotension (norepinephrine) — shock typically improves rapidly with steroids
- Treat precipitant: antibiotics for infection, etc.
Key Principles
- Do NOT delay steroids for diagnostic testing
- Hydrocortisone at stress doses provides both glucocorticoid AND mineralocorticoid activity (no need for separate fludrocortisone in acute phase)
- Hypotension refractory to fluids and vasopressors in a critically ill patient → think adrenal crisis
- Correct electrolytes (but hyperkalemia usually resolves with hydrocortisone and fluids)
Taper
- Once stable: taper to maintenance over 2-4 days
- Maintenance: hydrocortisone 15-25 mg/day (divided doses)
- Primary AI also needs fludrocortisone 0.05-0.1 mg PO daily (mineralocorticoid replacement)
Prevention
- Medical alert bracelet for all patients with adrenal insufficiency
- Sick day rules: double or triple oral glucocorticoid dose during illness
- Injectable hydrocortisone at home for emergencies (patient education)
- Stress dosing prior to surgery: hydrocortisone 100 mg IV before induction
Disposition
- ICU admission for hemodynamic instability or altered mental status
- Monitored bed for less severe presentations
- Endocrinology consultation
- Serial electrolytes, glucose monitoring
- Patient and family education on stress dosing before discharge
Calculators
Steroid Conversion Calculator
| Steroid Conversion Calculator | |
|---|---|
| Starting Steroid | 5 |
| Dose of Starting Steroid (mg) | 10 |
| Equivalent Doses | |
| Hydrocortisone (mg) | |
| Prednisone / Prednisolone (mg) | |
| Methylprednisolone (mg) | |
| Dexamethasone (mg) | |
| Relative Potency Reference | ||
|---|---|---|
| Steroid | Equivalent Dose (mg) | Relative Anti-inflammatory Potency |
| Hydrocortisone | 20 | 1 |
| Prednisone / Prednisolone | 5 | 4 |
| Methylprednisolone | 4 | 5 |
| Triamcinolone | 4 | 5 |
| Dexamethasone | 0.75 | 25-30 |
| References |
|---|
|
See Also
References
- ↑ Hahner S, et al. Adrenal crisis: prevalence, prevention, and education. Endocr Connect. 2015;4(2):R27-R35. PMID 25766587
- Bornstein SR, et al. Diagnosis and treatment of primary adrenal insufficiency: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2016;101(2):364-389. PMID 26760044
- Rushworth RL, et al. Adrenal crisis. N Engl J Med. 2019;381(9):852-861. PMID 31461595
- Puar TH, et al. Adrenal crisis: still a deadly event in the 21st century. Am J Med. 2016;129(3):339.e1-e9. PMID 26524708