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

From the Guidelines

Chronic primary adrenal insufficiency (Addison's disease) should be treated with lifelong hormone replacement therapy, primarily with glucocorticoids and often mineralocorticoids, with a typical regimen of 15-25 mg of hydrocortisone (HC) daily in split doses, and 50-200 µg of fludrocortisone as a single daily dose. The treatment of chronic primary adrenal insufficiency involves replacing the deficient hormones with oral medications, with the goal of mimicking the body's natural hormone production and minimizing symptoms and complications 1. Key aspects of treatment include:

• Glucocorticoid replacement with hydrocortisone (HC), with a daily dose of 15-25 mg, divided into 2-3 doses, and taken at specific times of the day to mimic the natural cortisol rhythm 1
• Mineralocorticoid replacement with fludrocortisone, with a daily dose of 50-200 µg, taken as a single dose 1
• Patient education on stress dosing, emergency management, and medication adherence to prevent life-threatening adrenal crisis 1
• Regular monitoring of symptoms, blood pressure, electrolytes, and plasma renin activity to optimize treatment 1
• Dose adjustments during illness, surgery, or significant stress, typically doubling or tripling the usual dose temporarily 1
• Carrying emergency injectable hydrocortisone (100 mg) and wearing medical alert identification for stress situations when higher doses are needed 1.

From the FDA Drug Label

DOSAGE & ADMINISTRATION Dosage depends on the severity of the disease and the response of the patient. ... 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. Fludrocortisone acetate tablets are preferably administered in conjunction with cortisone (10 mg to 37. 5 mg daily in divided doses) or hydrocortisone (10 mg to 30 mg daily in divided doses).

The treatment for chronic primary adrenal insufficiency (Addison's disease) is a combination of fludrocortisone and a glucocorticoid such as hydrocortisone or cortisone. The usual dose of fludrocortisone is 0.1 mg daily, and the dose of glucocorticoid ranges from 10 mg to 37.5 mg daily for cortisone and 10 mg to 30 mg daily for hydrocortisone, both in divided doses 2.

From the Research

Treatment Overview

The treatment for chronic primary adrenal insufficiency (Addison's disease) typically involves glucocorticoid replacement therapy, usually with hydrocortisone, and mineralocorticoid replacement therapy, usually with 9α-fluor-hydrocortisone (fludrocortisone) 3, 4.

Glucocorticoid Replacement Therapy

• The standard treatment regimen involves twice- or thrice-daily dosing with a glucocorticoid, most commonly oral hydrocortisone 5, 6.
• The dosage of hydrocortisone is usually 15-25 mg/day 3.
• In children with primary adrenal insufficiency, it is recommended to start glucocorticoid replacement therapy with three or four doses of hydrocortisone and adjust according to individual need 7.
• Long-acting glucocorticoids such as prednisolone and dexamethasone are not recommended in children with adrenal insufficiency 7.

Mineralocorticoid Replacement Therapy

• Patients with primary adrenal insufficiency usually require mineralocorticoid replacement therapy to compensate for the impaired aldosterone secretion 4.
• The standard replacement consists of 9α-fluorocortisol (fludrocortisone) given once daily as a single oral dose (0.05-0.2 mg) 4.
• Monitoring of mineralocorticoid replacement consists of clinical assessment and measurement of plasma renin activity (PRA) aiming at a PRA level in the upper normal range 4.

Additional Considerations

• In stressful situations and in febrile disorders, the glucocorticoid dosage must be increased prophylactically to prevent an "adrenal crisis" 3, 7.
• Most women with adrenal insufficiency will profit from the additional substitution of dehydroepiandrosterone (DHEA) with regard to well-being and sexual function 3.
• Patients with adrenal insufficiency should be monitored regularly, at least every three to four months, with clinical evaluation including weight gain, growth rate, blood pressure, and general well-being of the patient 7.