Addison’s disease (a primary adrenal insufficiency) is the loss or degradation of the adrenal cortex resulting in glucocorticoid and mineralocorticoid deficiency. Signs and symptoms include weakness, weight loss, joint pain, postural hypotension, headache, anorexia, constipation, diarrhea, hyperpigmentation, hyponatremia, hyperkalemia, hypoglycemia, and hypercalcemia. The most common culprit in the United States is an autoimmune process, but tuberculosis is the most common cause worldwide. Other causes include sarcoidosis, histoplasmosis, amyloidosis, metastatic malignancy, and adrenal hemorrhage.
The stress of surgery in the setting of a patient with Addison’s disease can precipitate an “Addisonian crisis” or “adrenal crisis.” This is similar to patients that have been on chronic steroid replacement therapy for a long time and stop taking their glucocorticoid. Signs and symptoms include low blood pressure, nausea and vomiting, syncope, hypoglycemia, hyponatremia, hyperkalemia, a metabolic acidosis, lethergy, confusion, and convulsions.
Preparation of such a patient for surgery should include treatment of hypovolemia, hyperkalemia, and hyponatremia. It is also recommended to give a stress dose of glucocorticoids (current literature suggests 100 mg of hydrocortisone phosphate IV every 24 hours is adequate). Note that this is less than the 200-300 mg historically recommended.
Treatment of Primary Adrenal Insufficiency
- Pharmacologic: hydrocortisone 100 mg IV q24h
- Physiologic: treatment of hypovolemia, hyperkalemia, and hyponatremia