Primary Adrenal Insufficiency (Addison Disease, PAI)

Give the stress dose - hydrocortisone 100 mg IV every 24 hours covers surgical stress in primary adrenal insufficiency; current literature supports this over the historical 200 to 300 mg. Confirm the home glucocorticoid and fludrocortisone were taken, and never let chronic steroids stop abruptly around surgery.

Optimize the chemistry before induction - basic metabolic panel and CBC. Chronic primary disease classically shows anemia, hyponatremia, and hyperkalemia, often with hypoglycemia. Treat hypovolemia, hyperkalemia, and hyponatremia preoperatively instead of chasing them after the incision.

Skip etomidate - etomidate sits on the list of drugs that cause adrenal insufficiency by suppressing cortisol synthesis. Pick another induction agent in a patient who already cannot mount a cortisol response.

Refractory shock means crisis - hypotension that will not answer fluids and vasopressors in a patient with known or suspected adrenal insufficiency is an adrenal crisis until proven otherwise. Give hydrocortisone immediately. Draw ACTH, cortisol, aldosterone, renin, and a chemistry panel with glucose only if it costs no delay, and do not run an ACTH stimulation test in the acute setting.

Watch the glucose - cortisol deficiency wrecks glucose homeostasis, and recurrent hypoglycemia is part of the disease; children can present with hypoglycemic seizures. Check point-of-care glucose intraoperatively and in PACU.

Look at the potassium before succinylcholine - hyperkalemia is one of the most common findings in chronic primary adrenal insufficiency. Read the day-of chemistry rather than assuming the patient is at their usual baseline.

Crisis looks like nothing specific - weakness, nausea and vomiting, abdominal or back pain, fever, tachycardia, confusion, obtundation. As many as half of patients go to shock without preceding hypotension, so a soft pressure in a steroid-dependent patient deserves treatment, not observation.

Know why they are deficient - drugs matter here. Ketoconazole, fluconazole, and metyrapone impair cortisol production; phenytoin and rifampin accelerate cortisol metabolism and can tip a marginal patient into crisis.

Postoperative period is still risky - adrenal crisis is precipitated by internal or external stress, and infection is the most frequent trigger. Hand off the steroid plan explicitly and flag unexplained postoperative hypotension, vomiting, or altered mental status as crisis until disproven.

Primary adrenal insufficiency (PAI) is destruction or dysfunction of the adrenal cortex itself, so both glucocorticoid and mineralocorticoid output fail. That is the distinction from secondary and tertiary disease, where aldosterone is preserved because the renin-angiotensin system does not depend on the pituitary. Autoimmune adrenalitis is the usual cause in developed countries, with anti-21-hydroxylase antibodies in about 90% of patients; tuberculosis leads worldwide. Cortisol supports vascular tone, gluconeogenesis, and the stress response, while aldosterone holds sodium and volume, so losing both gives hyponatremia, hyperkalemia, hypoglycemia, anemia, orthostatic hypotension, and salt craving, with hyperpigmentation from the ACTH drive.

The gland is usually about 90% destroyed before anything shows, so the disease stays masked until a stressor — infection, trauma, or surgery — unmasks it as adrenal crisis.