How is Addison's disease managed?

From the Guidelines

Addison's disease is managed primarily through lifelong hormone replacement therapy, with the most recent and highest quality study recommending hydrocortisone 15-20 mg in divided doses and fludrocortisone 0.05-0.1 mg/d, with adjustments based on clinical assessment and patient education on stress dosing and emergency injectables 1.

Management Approach

The management of Addison's disease involves:

• Daily oral glucocorticoids, such as hydrocortisone, to replace cortisol
• Mineralocorticoids, such as fludrocortisone, to maintain sodium and potassium balance
• Increased glucocorticoid dose during times of illness, stress, or surgery
• Emergency injection kit containing hydrocortisone for severe illness or if oral medication cannot be taken
• Regular monitoring through blood tests to adjust medication dosages
• Patient education on recognizing adrenal crisis symptoms and stress dosing

Key Considerations

• The preferred choice of glucocorticoid treatment is hydrocortisone or cortisone acetate, with no studies showing one is superior to the other 1
• Patients should wear medical alert identification and receive education on stress dosing, emergency injectables, and recognizing adrenal crisis symptoms 1
• Dietary sodium intake should be adequate, especially during hot weather when salt loss increases

Treatment Adjustments

• Treatment adjustments should be based on clinical assessment, including symptoms and signs of over-replacement or under-replacement 1
• Plasma ACTH and serum cortisol are not useful parameters for glucocorticoid dose adjustment, and monitoring relies on clinical assessment and patient education 1

Recent Guidelines

• The most recent guidelines recommend hydrocortisone 15-20 mg in divided doses and fludrocortisone 0.05-0.1 mg/d, with adjustments based on clinical assessment and patient education on stress dosing and emergency injectables 1
• The guidelines also emphasize the importance of patient education, medical alert identification, and regular monitoring to adjust medication dosages 1

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.

Management of Addison's Disease involves the use of fludrocortisone acetate tablets in combination with a glucocorticoid such as hydrocortisone or cortisone to provide substitution therapy approximating normal adrenal activity. The usual dose of fludrocortisone acetate tablets is 0.1 mg daily, with the possibility of adjusting the dosage based on the severity of the disease and the patient's response. This is according to 2.

From the Research

Management of Addison's Disease

Addison's disease is a rare disorder of the adrenal glands, and its management involves replacing the deficient hormones. The main goals of treatment are to replace the hormones cortisol and aldosterone, and to relieve the symptoms of the disease.

Replacement Therapy

• The hormone deficiencies in Addison's disease are conventionally treated with oral glucocorticoid and mineralocorticoid replacement 3, 4.
• Patients with Addison's disease require mineralocorticoid replacement, i.e., fludrocortisone 0.05 - 0.20 mg once daily 3.
• Starting doses of glucocorticoids should be 15 - 20 mg for hydrocortisone or 20 - 30 mg for cortisone acetate, divided into two or three doses, and preferentially weight-adjusted 3.
• Timed-release hydrocortisone tablets and continuous subcutaneous hydrocortisone infusion are promising new treatment modalities 3, 5.

Prevention of Addisonian Crisis

• In patients known to have adrenal insufficiency, inadequate adjustment of the glucocorticoid dosage during intercurrent illness can be life threatening 6.
• Patients with known adrenal insufficiency, as well as their relatives and general practitioners, should repeatedly receive verbal and written instructions on how to deal with physical and severe psychic stress 6.
• Treatment of an Addisonian crisis consists of immediate parenteral hydrocortisone (100 mg bolus) and subsequent continuous infusion until the underlying precipitating cause is cured and/or when regular supplementation can be continued 7.

Emerging Therapies

• New treatment modalities such as modified-released hydrocortisone and continuous subcutaneous hydrocortisone infusion have recently been developed, offering the potential for closer simulation of the physiological serum cortisol rhythm 5.
• Two studies have also looked at modifying the natural history of adrenal failure using adrenocorticotropic hormone (ACTH) stimulation and immunomodulatory therapies, leading to the concept of residual adrenal function in some Addison's disease patients 5.