What is the treatment for primary adrenal insufficiency (Addison's disease)?

From the Guidelines

The treatment for primary adrenal insufficiency (Addison's disease) involves lifelong hormone replacement therapy, with daily oral glucocorticoids, typically hydrocortisone (15-20 mg daily divided into 2-3 doses), and mineralocorticoid replacement with fludrocortisone (0.05-0.1 mg once daily) to maintain sodium and potassium balance, as recommended by the most recent guidelines 1.

Key Components of Treatment

• Daily oral glucocorticoids: hydrocortisone (15-20 mg daily divided into 2-3 doses) or prednisone (equivalent dose)
• Mineralocorticoid replacement: fludrocortisone (0.05-0.1 mg once daily)
• Dehydroepiandrosterone (DHEA) supplementation may be considered for women with low libido or poor energy despite adequate cortisol replacement
• Patients must carry emergency injectable hydrocortisone (100 mg) and wear medical alert identification for adrenal insufficiency

Adjusting Medication Dosages

• During illness, injury, or surgery, glucocorticoid doses need to be increased (typically doubled or tripled during minor illness with fever) 1
• Regular monitoring of blood pressure, electrolytes, and symptoms is essential to adjust medication dosages

Perioperative Management

• Hydrocortisone 100 mg by intravenous (i.v.) injection should be given at induction of anaesthesia, followed by a continuous infusion of hydrocortisone at 200 mg.24 h-1, until the patient can take double their usual oral glucocorticoid dose by mouth 1
• The period of fasting should be minimised and adrenal insufficient patients should be prioritised on routine surgical operating lists

Importance of Treatment

This replacement therapy is critical because the adrenal glands in Addison's disease cannot produce sufficient cortisol and aldosterone, hormones that regulate metabolism, stress response, blood pressure, and electrolyte balance. Without proper treatment, adrenal crisis—a life-threatening emergency—can occur during times of physical stress.

From the FDA Drug Label

DOSAGE & ADMINISTRATION Dosage depends on the severity of the disease and the response of the patient. Patients should be continually monitored for signs that indicate dosage adjustment is necessary, such as remission or exacerbations of the disease and stress (surgery, infection, trauma) (see WARNINGSand PRECAUTIONS, General) Addison's Disease In Addison’s disease, the combination of fludrocortisone acetate tablets with a glucocorticoid such as hydrocortisone or cortisone provides substitution therapy approximating normal adrenal activity with minimal risks of unwanted effects. The usual dose is 0.1 mg of fludrocortisone acetate tablets daily, although dosage ranging from 0.1 mg three times a week to 0. 2 mg daily has been employed. INDICATIONS & USAGE Fludrocortisone acetate tablets, 0.1 mg are indicated as partial replacement therapy for primary and secondary adrenocortical insufficiency in Addison’s disease and for the treatment of salt-losing adrenogenital syndrome.

The treatment for primary adrenal insufficiency (Addison's disease) is a combination of fludrocortisone acetate tablets with a glucocorticoid such as hydrocortisone or cortisone, providing substitution therapy that approximates normal adrenal activity with minimal risks of unwanted effects. The usual dose of fludrocortisone acetate tablets is 0.1 mg daily 2. This treatment is indicated as partial replacement therapy for primary and secondary adrenocortical insufficiency in Addison’s disease 2.

• The dosage may vary depending on the severity of the disease and the patient's response.
• Patients should be continually monitored for signs that indicate dosage adjustment is necessary.
• The treatment should be administered in conjunction with cortisone (10 mg to 37.5 mg daily) or hydrocortisone (10 mg to 30 mg daily) 2.

From the Research

Treatment Overview

The treatment for primary adrenal insufficiency, also known as Addison's disease, involves replacing the deficient hormones, specifically mineralocorticoids and glucocorticoids 3, 4, 5.

Replacement Therapy

• Mineralocorticoids and glucocorticoids replacement is essential for patients with primary adrenal insufficiency 3, 4, 5.
• The goal of replacement therapy is to mimic the physiological pattern of cortisol secretion by the normal adrenal gland 6.
• Patients require lifelong hormone supplementation, and education on dose adaptation during physical and mental stress or emergency situations is crucial to prevent life-threatening adrenal crises 7.

Stress-Dose Glucocorticoids

• During times of stress, such as illness or invasive surgical procedures, stress-dose glucocorticoids are required because the destruction of the adrenal glands prevents an adequate physiologic response 3.
• Patients should be educated on how to adjust their medication doses during stressful situations to prevent adrenal crises 7.

Patient Education and Management

• Successful therapy is mainly based on adequate patient education as a cornerstone in the prevention and management of adrenal crisis 4.
• Patients with adrenal insufficiency should carry an emergency card and emergency kit with them 7.
• Management of primary adrenal insufficiency requires vigilance for concomitant autoimmune diseases, as up to 50% of patients develop another autoimmune disorder during their lifetime 3.