Eduovisual
Endocrine
Adrenal crisis: stress-dose steroids and management
— Loss of cortisol → impaired vascular tone (loss of permissive effect on catecholamines), decreased gluconeogenesis, unopposed ADH → hyponatremia.
— Loss of aldosterone (primary disease only) → renal Na⁺ wasting, K⁺ and H⁺ retention → hyperkalemia, non-anion-gap metabolic acidosis, volume depletion.
— Known Addison disease, CAH, bilateral adrenalectomy, autoimmune polyglandular syndrome.
— Patients on chronic glucocorticoids ≥5 mg prednisone-equivalent for >3–4 weeks within the past year (HPA-axis suppression).
— Abrupt steroid discontinuation, missed doses during GI illness, or failure to stress-dose around surgery/sepsis.
— Pituitary apoplexy, Sheehan syndrome, recent pituitary surgery, brain irradiation.
— Bilateral adrenal hemorrhage: anticoagulated patient, meningococcemia (Waterhouse–Friderichsen), antiphospholipid syndrome, post-op state.
— New ICI (checkpoint inhibitor) therapy → hypophysitis or primary adrenalitis.
— Drug effects: etomidate (single dose can precipitate), ketoconazole, rifampin (↑cortisol clearance), phenytoin, mitotane.
Board pearl: In any vasopressor-resistant hypotensive patient with hyperpigmentation, vitiligo, or chronic steroid use — give hydrocortisone before the cosyntropin result returns. Diagnostic delay kills; empiric treatment does not.
— Profound fatigue, weakness, anorexia, weight loss (chronic), then acute decompensation.
— Nausea, vomiting, diffuse abdominal pain — can mimic acute surgical abdomen; unnecessary laparotomy is a classic miss.
— Fever (precipitating infection or cortisol-deficiency dysregulation).
— Confusion, lethargy, coma; seizures from hyponatremia or hypoglycemia.
— Myalgias, arthralgias, salt craving (primary disease).
— Exogenous steroid exposure: dose, duration, route (inhaled high-dose ICS, intra-articular, topical clobetasol, epidural injections all count), last dose, recent taper.
— Known autoimmune disease (Hashimoto, T1DM, vitiligo, premature ovarian insufficiency → APS-2).
— Anticoagulation, recent heparin (HIT with bilateral adrenal vein thrombosis), sepsis with purpura.
— Postpartum failure to lactate, amenorrhea → Sheehan.
— Headache + visual field defect + sudden hypotension → pituitary apoplexy.
— TB exposure, HIV, disseminated fungal infection, metastatic cancer (lung, breast, melanoma → adrenals).
— New oncology therapy: ipilimumab/nivolumab/pembrolizumab within weeks to months.
Key distinction: Primary adrenal insufficiency → hyperpigmentation (↑ACTH/POMC) + hyperkalemia + salt craving. Secondary/tertiary (pituitary or exogenous steroid) → pale skin, normal K⁺, no salt craving, but hypoglycemia and hyponatremia still occur. Both can crash into crisis — treatment in the acute setting is the same: hydrocortisone + fluids now, sort the etiology later.
— Hypotension — often orthostatic early, frank shock late; classically poorly responsive to crystalloid and norepinephrine until steroids are given.
— Tachycardia, narrow pulse pressure; fever or hypothermia.
— Tachypnea from metabolic acidosis (Kussmaul-like).
— Hyperpigmentation of palmar creases, knuckles, scars, buccal mucosa, areolae, gingival margins — primary disease, develops over months.
— Vitiligo patches (autoimmune association).
— Purpura fulminans, petechiae over flanks → suspect Waterhouse–Friderichsen or adrenal hemorrhage.
— Dry mucous membranes, decreased turgor (volume depletion).
— Diffuse tenderness, guarding, even rebound — pseudo-peritonitis. Pain often out of proportion to findings.
— Flank or back pain in adrenal hemorrhage.
CCS pearl: On the CCS case, after ABCs order "hydrocortisone 100 mg IV push" and "0.9% NS bolus 1 L" in the same clock advance as you order the cortisol and ACTH levels. Do not wait for labs. Then advance the clock 30–60 minutes and reassess BP, mental status, glucose, and potassium. If you advanced the clock without giving steroids, you will see hemodynamic deterioration scripted into the case.
— Random serum cortisol and plasma ACTH drawn before hydrocortisone (dexamethasone preferred if you want to preserve cosyntropin testing — it does not cross-react with the cortisol assay).
— BMP: expect hyponatremia, hyperkalemia, low bicarbonate, elevated BUN/Cr (prerenal).
— Glucose — hypoglycemia especially in children and secondary AI.
— CBC: eosinophilia and lymphocytosis (cortisol normally suppresses both); neutropenia variable.
— Calcium — mild hypercalcemia in ~6%.
— TSH, free T4 (concurrent autoimmune hypothyroidism; always replace cortisol before thyroid hormone to avoid precipitating crisis).
— Lactate, VBG, lipase, LFTs, UA, blood cultures, lactate, β-hCG in reproductive-age women.
— ECG: peaked T waves, widened QRS from hyperkalemia.
— Random cortisol <5 µg/dL in an acutely ill, hypotensive patient = diagnostic of adrenal insufficiency.
— Cortisol >18–20 µg/dL in that setting argues strongly against it.
— Intermediate values → cosyntropin stimulation after stabilization.
— High ACTH (>2× upper limit) + low cortisol = primary adrenal insufficiency.
— Low or inappropriately normal ACTH + low cortisol = secondary/tertiary.
— Suspect adrenal hemorrhage (anticoagulated, septic, flank pain, dropping H/H): non-contrast CT abdomen — bilateral adrenal enlargement with hyperdensity.
— Suspect pituitary cause (headache, visual changes, postpartum): MRI pituitary with contrast.
— TB workup if endemic exposure: CXR, IGRA, adrenal calcifications on CT.
Board pearl: The triad hyponatremia + hyperkalemia + hypoglycemia in a shocked patient is adrenal crisis until proven otherwise — even before you see hyperpigmentation. Hyperkalemia plus hyponatremia narrows the differential dramatically (the other big one is hypoaldosteronism/T4 RTA, which won't shock you).
— Measure baseline cortisol, give 250 µg cosyntropin IV/IM, recheck cortisol at 30 and 60 min.
— Peak cortisol <18 µg/dL (or <500 nmol/L) = adrenal insufficiency.
— Defer if patient is in active crisis; use dexamethasone 4 mg IV for treatment in the interim (does not interfere with the cortisol assay) and stim test once stable.
— In acute secondary AI (e.g., recent pituitary surgery <4–6 weeks), adrenals have not yet atrophied and may still respond — stim test can be falsely normal; use insulin tolerance test or metyrapone, or rely on clinical context + low ACTH.
— Primary: ↑ACTH, ↑renin, ↓aldosterone, +21-hydroxylase antibodies (autoimmune adrenalitis, most common US cause).
— Other primary etiologies: adrenal TB (CT calcifications), HIV-related (CMV adrenalitis, disseminated histo/cocci), metastases, infiltrative (amyloid, hemochromatosis), adrenoleukodystrophy (young male + neurologic findings → VLCFA levels), bilateral hemorrhage.
— Secondary: pituitary MRI, full anterior pituitary panel (TSH/free T4, LH/FSH, prolactin, GH/IGF-1).
— APS-2 (adult): Addison + autoimmune thyroid ± T1DM — check TPO Ab, TSH, A1c, B12.
— APS-1 (child): Addison + hypoparathyroidism + chronic mucocutaneous candidiasis — AIRE gene.
Step 3 management: When you suspect crisis but want to preserve diagnostics, give dexamethasone 4 mg IV instead of hydrocortisone, draw cortisol/ACTH, then perform cosyntropin stim test within 24 hours once perfusion is restored. After confirmation, switch to hydrocortisone for mineralocorticoid coverage at stress doses.
— (1) Replace cortisol — hydrocortisone 100 mg IV bolus immediately.
— (2) Restore intravascular volume and glucose — 1 L 0.9% NaCl with D5 wide open, then 2–3 L over the first 1–2 hours guided by BP and JVP.
— Hemodynamically unstable + clinical suspicion → empiric hydrocortisone 100 mg IV q6h + aggressive fluids + ICU.
— Stable but high pretest probability → draw cortisol/ACTH, give dexamethasone 4 mg IV if you want to preserve stim test, then formal evaluation.
— Known AI patient with intercurrent illness, vomiting → IM hydrocortisone 100 mg from emergency kit at home → ED.
— Minor illness/febrile (>38°C), minor procedure under local: double or triple oral hydrocortisone × 2–3 days ("sick-day rules").
— Moderate stress (cholecystectomy, hernia repair, pneumonia): hydrocortisone 50 mg IV q8h × 24 h, taper to baseline over 2–3 days.
— Major stress (cardiac surgery, sepsis, trauma, labor): hydrocortisone 100 mg IV bolus, then 200 mg/24 h (50 mg q6h or continuous infusion) × 24 h, taper as clinically improving.
— Crisis: 100 mg IV q6h (or 200 mg/24 h infusion) until stable, then taper to 2–3× oral maintenance and back to baseline over days.
— At hydrocortisone doses ≥50 mg/day, intrinsic mineralocorticoid activity is sufficient — hold fludrocortisone until daily HC ≤50 mg.
Board pearl: Treat first, test later. A normal cortisol drawn after steroid administration cannot rule out the diagnosis, but a dead patient cannot be treated.
— Glucocorticoid + mineralocorticoid activity at high doses.
— Dose: 100 mg IV bolus, then 50 mg IV q6h or 200 mg continuous infusion over 24 h.
— Taper by ~50% per day as clinical status improves; transition to oral when tolerating PO.
— Maintenance after recovery: 15–25 mg/day PO in 2–3 divided doses (largest on waking) or once-daily modified-release hydrocortisone (Plenadren).
— Dexamethasone 4 mg IV q12h — useful when stim test pending (no assay cross-reactivity); minimal mineralocorticoid activity, so add fluids and watch K⁺.
— Methylprednisolone 40 mg IV q12h — second-line; minimal mineralocorticoid effect.
— Equivalency: hydrocortisone 20 mg = prednisone 5 mg = methylprednisolone 4 mg = dexamethasone 0.75 mg.
— 0.9% NS with D5 (D5NS) at 1 L over first hour, then 4–6 L in first 24 h titrated to BP, UOP >0.5 mL/kg/h, mental status.
— Avoid hypotonic fluids initially despite hyponatremia — correct Na⁺ slowly (≤8–10 mEq/L per 24 h) to avoid osmotic demyelination, especially in chronic hyponatremia.
— Add 0.05–0.2 mg PO daily once hydrocortisone <50 mg/day in primary AI.
— Not needed in secondary AI (RAAS preserved).
— Norepinephrine first-line if MAP <65 after steroid + fluids; vasopressin adjunct. Shock is typically steroid-responsive within 4–6 hours.
Step 3 management: Do not give etomidate for intubation in adrenal-insufficient or septic patients — single dose suppresses 11β-hydroxylase for up to 24 h. Use ketamine or propofol (with pressor support) for RSI.
— Two large-bore IVs or central line if pressor-requiring; arterial line for shock.
— Foley to track UOP.
— Avoid etomidate; rocuronium + ketamine is a good RSI combo when intubation needed.
— Minor (dental, cataract, colonoscopy w/o GA): usual morning HC dose + extra 25–50 mg HC IV at induction; resume baseline next day.
— Moderate (lap chole, joint replacement, vaginal delivery): HC 50 mg IV at induction + 25 mg IV q8h × 24 h, then taper.
— Major (open cardiothoracic, Whipple, liver transplant, cesarean): HC 100 mg IV at induction + 50 mg IV q6h × 24 h, taper over 2–3 days.
— Labor: HC 100 mg IV at onset of active labor, then 50 mg q6h until delivery; double oral dose × 24–48 h post-delivery.
— Trauma/critical illness: treat as major stress.
— Any patient who has received ≥5 mg prednisone/day for ≥3–4 weeks in the prior year is presumed HPA-suppressed and needs perioperative stress dosing.
— Inhaled fluticasone ≥1 mg/day, high-dose intranasal, frequent intra-articular injections — also count.
— Reverse anticoagulation, treat sepsis, lifelong steroid replacement assumed.
— Surgical decompression rarely indicated; supportive care + endocrine follow-up.
— Cortisol-clearance inducers: rifampin, phenytoin, phenobarbital, carbamazepine, St. John's wort → may need 2× hydrocortisone dose.
— Cortisol-synthesis inhibitors: ketoconazole, fluconazole (high-dose), etomidate, mitotane, metyrapone, osilodrostat.
— Mineralocorticoid antagonists: spironolactone, eplerenone — worsen hyperkalemia/hypotension.
CCS pearl: When a stem says "patient on chronic prednisone for RA presenting for emergent appendectomy," your orders should include hydrocortisone 100 mg IV preoperatively alongside antibiotics and IV fluids — missing this is a scripted deterioration.
— Higher baseline mortality from crisis; often present atypically with delirium and falls rather than classic GI/skin findings.
— Polypharmacy: review for steroid-suppressing meds (inhaled, topical, intra-articular), opioids masking abdominal pain, antihypertensives worsening hypotension.
— Comorbid CHF/CKD complicates aggressive fluid resuscitation — still resuscitate, but monitor with bedside ultrasound, frequent reassessment, and consider continuous HC infusion over boluses to reduce sodium load.
— Beware concurrent infection — UTI, pneumonia, C. difficile are top precipitants; cover empirically.
— Adrenal autoimmunity is less common in elderly new diagnoses; consider metastatic disease, bilateral hemorrhage (often on DOAC/warfarin), TB reactivation, drug-induced (checkpoint inhibitor use is rising in this demographic).
— Hyperkalemia is more severe and slower to correct; have dialysis on standby for K⁺ >6.5 with ECG changes refractory to medical therapy.
— Hydrocortisone clearance largely hepatic — no renal dose adjustment required.
— Volume status assessment harder; use POCUS IVC, lung B-lines.
— Cortisol metabolism reduced — paradoxically these patients may have lower effective requirements, but in crisis still give full stress dose.
— Albumin low → free cortisol fraction higher; total cortisol levels may misleadingly appear "low" — interpret with caution. Salivary cortisol or free cortisol index more accurate in cirrhotics.
— Relative adrenal insufficiency in cirrhosis with septic shock ("hepatoadrenal syndrome") — consider hydrocortisone 200 mg/day if pressor-requiring.
— Glucose control worsens with stress dosing — anticipate insulin needs to double or triple; use sliding scale + basal adjustment.
Key distinction: In cirrhosis-related "relative AI," random cortisol may be normal but delta cortisol after cosyntropin <9 µg/dL suggests inadequate reserve — treat in the setting of refractory septic shock per Surviving Sepsis (HC 200 mg/day).
— Normal pregnancy raises CBG and total cortisol 2–3× by third trimester; use trimester-specific reference ranges or free cortisol.
— Diagnosis in pregnancy: morning cortisol <3 µg/dL strongly suggests AI; cosyntropin stim test is safe — use trimester-adjusted cutoffs (e.g., peak <25 µg/dL in third trimester suggests AI).
— Maintenance dosing typically increases ~20–40% in third trimester; fludrocortisone often unchanged but titrate to BP, K⁺, edema.
— Labor and delivery: treat as major surgical stress — HC 100 mg IV at active labor onset, then 50 mg IV q6h until 24–48 h postpartum, then taper to pre-pregnancy dose.
— Cesarean: HC 100 mg IV at induction, 50 mg q6h × 24–48 h.
— Postpartum new diagnosis: think Sheehan syndrome (failure to lactate, amenorrhea, fatigue).
— Neonatal CAH (21-hydroxylase deficiency) — classic presentation week 1–2 of life with vomiting, dehydration, hyponatremia, hyperkalemia, hypoglycemia, ambiguous genitalia in genetic females. Newborn screening detects most cases.
— Crisis management: HC 50 mg/m² IV bolus, then 50–100 mg/m²/day divided q6h; D10 NS bolus 20 mL/kg.
— Stress dosing for sick days: triple oral HC; for vomiting/inability to take PO → IM HC injection (parents trained at diagnosis).
— Avoid dexamethasone for chronic replacement in growing children (growth suppression).
— Always replace mineralocorticoid (fludrocortisone) plus salt supplementation in infants.
Board pearl: Female neonate with virilized external genitalia + salt-wasting crisis on day 7–14 → 21-hydroxylase deficiency CAH → confirm with elevated 17-hydroxyprogesterone; treat with HC + fludrocortisone + NaCl supplements lifelong.
— Refractory shock and death — leading cause of mortality.
— Arrhythmias from hyperkalemia (peaked T → wide QRS → sine wave → VF/asystole).
— Demand ischemia and type 2 MI in patients with CAD.
— Hypoglycemic seizures and anoxic brain injury, especially in children.
— Osmotic demyelination syndrome (central pontine myelinolysis) from overly rapid Na⁺ correction — limit to ≤8–10 mEq/L per 24 h (≤6 mEq/L if high risk: chronic hyponatremia, alcoholism, malnutrition, hypokalemia, liver disease). If overcorrection occurs, re-lower with D5W ± DDAVP.
— Cerebral edema in pediatric crisis with aggressive hypotonic fluid resuscitation.
— Status epilepticus from severe hyponatremia.
— Hyperkalemic cardiac arrest if untreated.
— Persistent hypoglycemia in pediatric and secondary AI.
— Lactic acidosis from shock and impaired gluconeogenesis.
— Steroid-induced hyperglycemia, psychosis, hypokalemia, fluid overload.
— Stress ulcers — consider PPI prophylaxis in ICU patients.
— Opportunistic infection unmasked by glucocorticoid (e.g., disseminated strongyloidiasis in endemic patients — screen and treat with ivermectin before high-dose steroids when feasible).
— Hospital-acquired pneumonia, VTE — DVT prophylaxis is essential.
— Recurrent crises (5–10/100 patient-years in known AI) — most from missed sick-day dosing.
— Reduced quality of life, fatigue, fertility issues, osteoporosis from cumulative steroid use.
— Adrenal hemorrhage survivors → lifelong primary AI.
Key distinction: Hyperkalemia in crisis usually resolves with fluids + hydrocortisone alone; reflexively treating with insulin/dextrose in an already-hypoglycemic patient can be disastrous. Always check glucose before insulin.
— Vasopressor requirement, persistent MAP <65 after initial resuscitation.
— Altered mental status, seizures, or coma.
— Severe hyperkalemia (K⁺ >6.5) or ECG changes.
— Severe hyponatremia (<120) requiring controlled correction.
— Refractory hypoglycemia.
— Mechanical ventilation, ongoing GI losses, or unstable precipitant (sepsis, MI, GI bleed).
— Endocrinology — every confirmed adrenal crisis; mandatory for new diagnoses, dose adjustments, and discharge education.
— Infectious disease — atypical infections (TB, fungal, HIV-related), septic precipitants.
— Critical care — distributive shock unresponsive to initial therapy.
— Neurosurgery — pituitary apoplexy requiring decompression (visual loss or deteriorating consciousness).
— Oncology — ICI-induced hypophysitis/adrenalitis; need to coordinate continuation of immunotherapy.
— Surgery — only if true intra-abdominal pathology after steroid response (most "abdomens" resolve within 12 h).
— Known Addison patient with febrile illness + vomiting but stable vitals → ED for IV HC + fluids + observation 6–12 h; safe discharge if tolerating PO and stable.
— Pituitary apoplexy → emergent MRI, ICU, neurosurgery, ophthalmology.
— Adrenal hemorrhage on anticoagulation → ICU, reverse anticoagulation, hematology.
CCS pearl: When you advance the clock and the patient remains hypotensive despite HC + 3 L NS + norepinephrine, your next orders should be vasopressin, lactate recheck, cortisol-axis verification (was HC actually given on time?), and look for an untreated precipitant — usually occult sepsis, MI, or GI bleed.
— Myxedema coma: hypothermia, hypoventilation, bradycardia, hyponatremia, hypoglycemia, altered mental status. Coexists with adrenal insufficiency in ~5–10% — give hydrocortisone before levothyroxine to avoid precipitating crisis. Confirm with TSH, free T4.
— Thyroid storm: tachycardia, fever, agitation, AF — opposite picture; but coexisting AI possible in autoimmune polyglandular disease.
— DKA/HHS: hyperglycemia (vs hypoglycemia in AI), Kussmaul breathing, ketonemia — distinguishes easily on labs; however, infection can precipitate both simultaneously.
— Hypoglycemia from insulinoma or sulfonylurea: isolated hypoglycemia without hyperkalemia or hyponatremia; check C-peptide and sulfonylurea screen.
— Pheochromocytoma crisis: paroxysmal hypertension, headache, palpitations — opposite hemodynamic picture, but can present with shock after catecholamine depletion or hemorrhage.
— Hypopituitarism (panhypopituitarism): broader endocrine deficiency — same acute management (steroids first) plus thyroid, GH, gonadotropin replacement.
— SIADH: hyponatremia with euvolemia, normal K⁺, normal cortisol, low uric acid — no hemodynamic compromise.
— Cerebral salt wasting: hyponatremia + hypovolemia + high urine Na⁺ in neurosurgical patient — looks similar electrolyte-wise but cortisol intact.
— Type 4 RTA (hyporeninemic hypoaldosteronism): hyperkalemia + non-AG acidosis in diabetic CKD patient — chronic, not shock.
Key distinction: If hyponatremia + hyperkalemia coexist with shock and hypoglycemia, it is adrenal crisis until proven otherwise. SIADH never causes hyperkalemia; type 4 RTA never causes shock; CSW never causes hyperkalemia.
— Same distributive pattern; can be the precipitant of crisis or stand alone. In refractory septic shock requiring pressors, give hydrocortisone 200 mg/day per Surviving Sepsis regardless of known AI status — improves shock resolution.
— GI losses, hemorrhage — responds well to fluids/blood; no hyperkalemia/hypoglycemia unless concurrent AI.
— Cool extremities, elevated JVP, pulmonary edema; ECG/troponin/echo distinguish.
— Acute onset, urticaria, bronchospasm, exposure history; epinephrine first-line — steroids are adjunctive.
— Appendicitis, perforation, pancreatitis, mesenteric ischemia, ectopic pregnancy — imaging differentiates. Always give hydrocortisone before laparotomy in any patient on chronic steroids or with known AI.
— Gastroenteritis, bowel obstruction, DKA — labs and history clarify; remember GE is the most common precipitant of crisis in known AI.
— Beta-blocker, calcium-channel-blocker overdose → bradycardia + hypotension + hyperkalemia + hypoglycemia (CCB) — can mimic AI; check med list, give glucagon/calcium/insulin-euglycemic.
— Opioid overdose: respiratory depression, miosis.
— Spinal cord injury, bradycardia + hypotension; history of trauma.
— Beer potomania, tea-and-toast diet, psychogenic polydipsia — no hyperkalemia, no hypotension.
— Liver failure, sepsis, sulfonylurea, alcoholic ketoacidosis — context-driven.
Board pearl: Vasopressor-refractory shock + eosinophilia is adrenal crisis until proven otherwise. Eosinophilia is a free clue normally suppressed by cortisol; its presence in a shocked patient is highly specific.
— Off IV pressors and fluids ≥24 h.
— Tolerating oral hydrocortisone and fludrocortisone (if primary) with stable BP and K⁺.
— Precipitant identified and addressed (antibiotics completed or transitioned).
— Education completed and emergency kit dispensed.
— Hydrocortisone 15–25 mg/day PO in 2–3 divided doses (largest dose on waking, e.g., 10–10–5 mg or 15–10 mg). Alternative: once-daily modified-release HC.
— Fludrocortisone 0.05–0.2 mg PO daily for primary AI; titrate to normal BP (no orthostasis), normal K⁺, plasma renin in upper-normal range.
— Increase sodium intake in hot weather/exercise.
— Rule of 3s/2s: Fever >38°C or moderate illness → double or triple usual oral dose for 2–3 days.
— Vomiting or unable to keep down pills → inject IM hydrocortisone 100 mg from emergency kit and go to ED.
— Minor surgery/dental → extra 25–50 mg HC at time of procedure.
— Major surgery/trauma/labor → IV stress dosing protocol.
— Hydrocortisone 100 mg vial + diluent + syringe/needles (or Solu-Cortef Act-O-Vial).
— Written instructions, glucagon (for pediatric patients).
— MedicAlert bracelet/necklace indicating "adrenal insufficiency — steroid-dependent."
— Wallet card with diagnosis, regimen, emergency contact, and endocrinologist.
Step 3 management: Every patient discharged after adrenal crisis must leave with (1) prescription for oral HC, (2) prescription for fludrocortisone if primary, (3) emergency IM HC kit, (4) MedicAlert ordered, (5) endocrinology follow-up within 2–4 weeks. Missing any of these is a frequent malpractice and Step 3 vignette setup.
— Endocrinology within 2–4 weeks of discharge; then every 3–6 months until stable, then annually.
— Primary care within 1–2 weeks for medication reconciliation and adherence.
— Clinical: weight, BP (supine + standing), symptoms of over/under-replacement (Cushingoid vs fatigue/weight loss/postural symptoms).
— Labs:
– Electrolytes and glucose every 3–6 months (Na⁺, K⁺).
– Plasma renin activity — guides fludrocortisone dose (target upper-normal range).
– ACTH levels are not used to titrate replacement (often remain high in primary AI).
– Annual TSH, fasting glucose, lipids, A1c (steroid-related dysmetabolism).
— DEXA at diagnosis and every 2 years; supplement calcium 1000–1200 mg and vitamin D 800–1000 IU; bisphosphonate if osteoporosis.
— Screen for associated autoimmunity: annual TSH, B12, A1c, celiac antibodies as indicated (APS-2).
— Sick-day rules reviewed at every visit — most crises are preventable.
— Travel: carry 2× supply, prescriptions, emergency kit in carry-on; jet-lag dose timing.
— Pregnancy planning: pre-conception counseling, dose adjustment in third trimester, peripartum stress dosing.
— Exercise and sports: usually no dose change for routine activity; extreme endurance events may need 5–10 mg extra HC.
— Mental health: depression and anxiety more common; QoL questionnaires periodically.
Board pearl: Plasma renin (not aldosterone or ACTH) is the lab to follow for fludrocortisone titration in primary AI — high renin = need more fludrocortisone; suppressed renin + hypertension/hypokalemia = too much.
— Patient in active crisis with altered mental status — implied consent for emergent steroid administration and resuscitation; document clinical urgency.
— Religious refusal of steroids (rare) — explore alternatives only after explaining the near-certain fatality of untreated crisis; involve ethics committee if time permits; treat over objection only with court order in non-emergent presentations.
— Adrenal hemorrhage from suspected HIT requires adverse event reporting and heparin allergy documentation in the EHR.
— Suspected medication error (missed perioperative stress dose causing crisis) → root-cause analysis, disclosure to patient/family per institutional policy and state law.
— Discharge medication reconciliation failure → patient leaves without fludrocortisone or with wrong HC dose → readmission.
— Hand-off from ICU to ward: explicitly document HC taper schedule, last stress dose, and resumption-of-home-dose plan; verbal + written SBAR.
— Outpatient handoff: ensure endocrinology appointment booked before discharge, not just referred; closed-loop communication.
— Surgical scheduling: preoperative checklist must include "history of chronic steroid use" → triggers stress-dose protocol.
— MedicAlert is a safety device, not a nicety — order it before discharge.
— High-alert medication: hydrocortisone for crisis should be on automatic dispensing cabinet override list in ED/ICU.
— Avoid etomidate in known AI — document allergy/avoidance in EHR.
— Drug-interaction alerts for rifampin, phenytoin, ketoconazole in AI patients.
— Each prevented crisis admission saves ~$10–20K; structured patient education programs (group classes, telehealth check-ins) are reimbursable under chronic care management codes.
— Document shared decision-making for fludrocortisone vs salt-loading strategies in mild cases.
Key distinction: Failure to administer stress-dose steroids to a chronic-steroid patient undergoing surgery is one of the most frequently cited preventable perioperative deaths — institutional protocols, EHR alerts, and preoperative checklists are the standard of care.
— Hyponatremia + hyperkalemia + hypoglycemia + shock = adrenal crisis.
— Hyperpigmentation + vitiligo + autoimmune thyroid disease + Addison = APS-2.
— Hypoparathyroidism + mucocutaneous candidiasis + Addison = APS-1 (AIRE).
— Meningococcemia + purpura + shock + bilateral adrenal hemorrhage = Waterhouse–Friderichsen.
— Postpartum failure to lactate + amenorrhea + fatigue = Sheehan.
— Headache + bitemporal hemianopsia + hypotension = pituitary apoplexy.
— Young male + adrenal insufficiency + spastic paraparesis + behavioral change = X-linked adrenoleukodystrophy (VLCFAs).
— Primary: autoimmune adrenalitis (80%), TB (worldwide #1), infection (HIV/CMV, fungal), metastases, hemorrhage, drugs, infiltration.
— Secondary: exogenous steroid withdrawal (by far #1), pituitary tumor/surgery/radiation, Sheehan, hypophysitis (lymphocytic, IgG4, ICI-related).
— Etomidate — single-dose adrenal suppression up to 24 h.
— Ketoconazole, fluconazole, mitotane, metyrapone, osilodrostat — block steroidogenesis.
— Rifampin, phenytoin, phenobarbital, carbamazepine, St. John's wort — induce cortisol clearance → may need 2× HC dose.
— Opiates and megestrol — suppress HPA axis.
— Checkpoint inhibitors — primary adrenalitis and hypophysitis.
— Random cortisol <5 in shock = AI; >18 rules it out.
— Use dexamethasone to treat while preserving cosyntropin testing.
— Eosinophilia + hyperkalemia + hypoglycemia + hypotension = the "free diagnosis."
— HC 15–25 mg/day, fludro 0.05–0.2 mg/day, plasma renin upper-normal.
Board pearl: Always replace glucocorticoid before levothyroxine in coexisting AI + hypothyroidism — thyroid hormone accelerates cortisol clearance and precipitates crisis.
— Stem: known Addison, vomiting × 24 h, BP 80/40, K⁺ 6.0, Na⁺ 128, glucose 50. Answer: Hydrocortisone 100 mg IV + NS with D5 bolus (do not wait for cortisol).
— Stem: RA on prednisone 10 mg daily × 5 years, scheduled for hip replacement. Next step: stress-dose hydrocortisone 100 mg IV at induction, 50 mg IV q6h × 24 h, taper.
— Stem: pneumonia, MAP 60 on norepinephrine and vasopressin. Next step: add hydrocortisone 200 mg/day IV (Surviving Sepsis).
— Stem: anticoagulated for AF, sudden flank pain, hypotension, dropping hematocrit, hyperkalemia. Answer: CT abdomen + empiric hydrocortisone + reverse anticoagulation.
— Stem: macroadenoma patient, sudden headache, visual field defect, hypotension. Next step: hydrocortisone IV + MRI + neurosurgery consult.
— Stem: 10-day-old female, ambiguous genitalia, vomiting, Na⁺ 122, K⁺ 7.0. Answer: HC 50 mg/m² IV + D10 NS + fludrocortisone + NaCl; confirm with 17-OHP.
— Stem: suspected crisis but want to confirm. Answer: dexamethasone 4 mg IV (does not interfere with cortisol assay), draw cortisol/ACTH, then cosyntropin stim test.
— Stem: new myxedema picture with low TSH-axis labs. Order: hydrocortisone before levothyroxine.
— Best next step: MedicAlert + emergency IM HC kit + sick-day rules + endocrine follow-up in 2–4 weeks.
— Diagnosis: primary adrenal insufficiency; confirm with cosyntropin stim.
Step 3 management: When a question offers "draw labs first" vs "give steroids now" in a hypotensive patient with electrolyte derangement, steroids first is almost always correct.
Adrenal crisis is a steroid-deficient distributive shock that demands empiric IV hydrocortisone 100 mg + aggressive D5-NS resuscitation before any diagnostic confirmation, with simultaneous treatment of the precipitant and a structured stress-dose, sick-day, and discharge education plan to prevent recurrence.