What are the national guidelines for treating Addison's disease (Adrenal Insufficiency)?

From the Guidelines

The treatment of Addison's disease should follow a regimen of hormone replacement therapy, with oral hydrocortisone at a total daily dose of 15-25 mg divided into 2-3 doses, and fludrocortisone at a dose of 50-200 µg as a single daily dose, as recommended by the most recent guidelines 1. The cornerstone of treatment is glucocorticoid replacement, typically with oral hydrocortisone, to mimic the natural cortisol rhythm. Mineralocorticoid replacement is achieved with fludrocortisone, adjusted based on blood pressure, electrolytes, and plasma renin activity.

• Patients should also be advised to increase their salt intake, particularly in hot weather or during exercise.
• For acute adrenal crisis, immediate treatment with intravenous hydrocortisone (100 mg bolus followed by 100 mg 6-8 hourly) and fluid resuscitation with normal saline is essential, as stated in the guidelines 1.
• All patients must carry a steroid emergency card and medical alert identification, and should be educated on "sick day rules" - typically doubling or tripling their glucocorticoid dose during illness, injury, or significant stress.
• Regular monitoring includes clinical assessment of symptoms, blood pressure measurements, and periodic laboratory tests for electrolytes and renin levels, with patients being reviewed at least annually 1. This replacement approach is necessary because Addison's disease results from the adrenal glands' inability to produce sufficient cortisol and aldosterone, which are critical for maintaining energy, blood pressure, and electrolyte balance.
• The diagnosis of primary adrenal insufficiency should be considered in all patients presenting with unexplained collapse, hypotension, vomiting, or diarrhea, and hyperpigmentation, hyponatremia, hyperkalemia, acidosis, and hypoglycemia increase clinical suspicion of primary adrenal insufficiency 1.
• The aetiology of primary adrenal insufficiency should be ascertained starting with the measurement of serum 21-hydroxylase (anti-adrenal) autoantibodies, and if antibodies are negative, CT imaging is recommended 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.

The national guidelines for treating Addison's disease are not explicitly stated in the provided drug labels. However, the labels do provide information on the dosage and administration of fludrocortisone acetate tablets for Addison's disease, which includes:

• A usual dose of 0.1 mg daily
• Combination therapy with a glucocorticoid such as hydrocortisone or cortisone
• Dosage adjustments based on the severity of the disease and the response of the patient 2.

From the Research

National Guidelines for Treating Addison's Disease

The national guidelines for treating Addison's disease involve replacing the deficient hormones, including glucocorticoids and mineralocorticoids.

• The recommended daily starting dose for hydrocortisone is 15-20 mg, divided into two or three doses, and preferentially weight-adjusted 3, 4.
• The mineralocorticoid depletion should be treated with fludrocortisone 0.05-0.20 mg once daily 3 or 0.2 mg to maintain adequate sodium and water balance 5.
• Replacement of dehydroepiandrosterone (DHEA) 20-50 mg has been advocated in adrenal failure, but the evidence for benefit is weak 4.
• 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 6.

Treatment Modalities

Different treatment modalities are available for Addison's disease, including:

• Oral glucocorticoid and mineralocorticoid replacement 3, 4
• Timed-release hydrocortisone tablets 3
• Continuous subcutaneous hydrocortisone infusion 3, 6
• Replacement of DHEA or testosterone for selected groups of patients 3, 4

Diagnosis and Assessment

The diagnosis of Addison's disease involves measuring basal plasma cortisol and synthetic adrenocorticotropin 1-24 7.

• Elevated plasma levels of adrenocorticotropin and renin confirm the diagnosis 7.
• Plasma-renin activity can be used to assess sodium balance in patients with Addison's disease and their response to fludrocortisone 5.