Goals of the treatment are to limit morbidity and mortality, return the patient to a normal functional state, and prevent episodes of acute adrenal insufficiency.
Nonpharmacologic Therapy
Inform patients of treatment complications, expected outcomes, proper medication administration and adherence, and possible side effects.
Pharmacotherapy
Corticosteroids
Hydrocortisone, cortisone, and prednisone are the glucocorticoids of choice, administered twice daily at the lowest effective dose while mimicking the normal diurnal adrenal rhythm of cortisol production. Recommended starting total daily doses are hydrocortisone 15 to 25 mg daily, which is approximately equivalent to cortisone acetate 25 to 37.5 mg, or prednisone 2.5 mg. Two thirds of the dose is given in the morning, and one third is given 6 to 8 hours later. The patient’s symptoms can be monitored every 6 to 8 weeks to assess proper glucocorticoid replacement.
Fludrocortisone acetate 0.05 to 0.2 mg orally once daily can be used to replace mineralocorticoid loss. If parenteral therapy is needed, 2 to 5 mg of deoxycorticosterone trimethylacetate in oil can be administered intramuscularly every 3 to 4 weeks. The major reason for adding the mineralocorticoid is to minimize development of Hyperkalemia.
Because most adrenal crises occur because of glucocorticoid dose reductions or lack of stress-related dose adjustments, patients receiving corticosteroid replacement therapy should add 5 to 10 mg hydrocortisone (or equivalent) to their normal daily regimen shortly before strenuous activities, such as exercise. During times of severe physical stress (eg, febrile illnesses and after accidents), patients should be instructed to double their daily dose until recovery.
Treatment of secondary adrenal insufficiency is identical to primary disease treatment, with the exception that mineralocorticoid replacement is usually not necessary.
Pharmacotherapy of Acute Adrenal Insufficiency
Acute adrenal insufficiency (also known as adrenal crisis or addisonian crisis) represents a true endocrine emergency. Stressful situations, surgery, infection, and trauma are potential events that increase adrenal requirements, especially in patients with some underlying adrenal or pituitary insufficiency. The most common cause of adrenal crisis is abrupt withdrawal of exogenous glucocorticoids in patients receiving chronic treatment that resulted in hypothalamicpituitary- adrenal-axis suppression.
Hydrocortisone given parenterally is the corticosteroid of choice because of its combined glucocorticoid and mineralocorticoid activity. The starting dose is 100 mg IV by rapid infusion, followed by a continuous infusion (usually 10 mg/h) or intermittent bolus of 100 to 200 mg every 24 hours. IV administration is continued for 24 to 48 hours. If the patient is stable at that time, oral hydrocortisone can be started at a dose of 50 mg every 6 to 8 hours, followed by tapering to the individual’s chronic replacement needs.
Fluid replacement often is required and can be accomplished with IV dextrose 5% in normal saline solution at a rate to support blood pressure. If hyperkalemia is present after the hydrocortisone maintenance phase, additional mineralocorticoid supplementation can be achieved with fludrocortisone acetate 0.1 mg daily.
Patients with adrenal insufficiency should carry a card or wear a bracelet or necklace that contains information about their condition. They should also have easy access to injectable hydrocortisone or glucocorticoid suppositories in case of an emergency or during times of physical stress, such as febrile illness or injury.
