What is the treatment for Adrenal Insufficiency (Addison's disease)?

From the Guidelines

Treatment for adrenal insufficiency should prioritize hormone replacement therapy with daily oral hydrocortisone (15-25 mg divided into 2-3 doses) and fludrocortisone (0.05-0.2 mg once daily) for primary adrenal insufficiency, with adjustments for physical stress and monitoring of symptoms, blood pressure, and electrolytes to prevent adrenal crisis and ensure quality of life. The standard treatment includes daily oral hydrocortisone to replace cortisol, and in primary adrenal insufficiency, fludrocortisone to replace aldosterone 1. Patients should carry emergency injectable hydrocortisone (100 mg) and wear a medical alert bracelet. During times of physical stress such as illness, injury, or surgery, glucocorticoid doses need to be increased (typically doubled or tripled) 1. For minor illness with fever, the hydrocortisone dose should be doubled until recovery. For severe illness, vomiting, or inability to take oral medication, immediate medical attention is needed for parenteral hydrocortisone administration 1. Regular monitoring of symptoms, blood pressure, and electrolytes is essential to adjust medication doses. Proper treatment prevents adrenal crisis, which is life-threatening, and allows patients to lead normal lives. The goal is to mimic the body's natural hormone rhythm while avoiding overtreatment, which can lead to cushingoid symptoms and metabolic complications. Some key considerations in the management of adrenal insufficiency include:

• Education on stress dosing for sick days, use of emergency injectables, and a medical alert bracelet or necklace for adrenal insufficiency to trigger stress dose corticosteroids by emergency medical personnel 1
• Endocrine consultation should be part of planning before surgery or high-stress treatments 1
• Using hydrocortisone allows for recreation of the diurnal rhythm of cortisol, with 2/3 of the dose given in the morning and 1/3 in the early afternoon 1
• DHEA replacement is controversial but deficiency can be tested and replacement considered in women with low libido and/or energy who are judged to be otherwise well replaced 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. 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. ACTIONS Naturally occurring glucocorticoids (hydrocortisone and cortisone), which also have salt-retaining properties, are used as replacement therapy in adrenocortical deficiency states.

The treatment for Adrenal Insufficiency involves replacement therapy with glucocorticoids such as hydrocortisone or cortisone, and mineralocorticoids such as fludrocortisone. The dosage of fludrocortisone depends on the severity of the disease and the response of the patient, with a usual dose of 0.1 mg daily for Addison's disease. The combination of fludrocortisone with a glucocorticoid provides substitution therapy approximating normal adrenal activity with minimal risks of unwanted effects 2. Fludrocortisone is indicated as partial replacement therapy for primary and secondary adrenocortical insufficiency in Addison’s disease 2. Hydrocortisone is used as replacement therapy in adrenocortical deficiency states 3.

• The treatment should be individualized and monitored for signs that indicate dosage adjustment is necessary.
• The treatment should be used with caution and under the guidance of a healthcare professional.

From the Research

Adrenal Insufficiency Treatment Overview

• Adrenal insufficiency is a rare but life-threatening disease that requires life-long glucocorticoid substitution therapy 4, 5, 6.
• Conventional therapy consists of glucocorticoid replacement using hydrocortisone administered two or three times daily, but this therapy has been shown to have limitations in normalizing total cortisol exposure and responding to increased need for glucocorticoids during illness and stress 4, 5.

Current Treatment Strategies

• Modified-release preparations of hydrocortisone have been developed to more closely mimic the physiological circadian pattern of cortisol secretion than conventional twice or thrice daily treatment 4, 7.
• Continuous subcutaneous hydrocortisone infusion (CSHI) is a novel approach that permits individualized glucocorticoid delivery and mimics a more physiologic cortisol pattern compared with oral glucocorticoid therapy 8.
• CSHI has been shown to decrease the median total daily dose of glucocorticoid, reduce the number of ED visits and hospitalization days due to adrenal crisis, and improve subjective health scores 8.

Treatment Goals and Recommendations

• The goal of treatment is to reduce the signs and symptoms of the disease, prevent the development of an addisonian crisis, and improve quality of life 6, 7.
• Treatment should aim to mimic the normal circadian profile of cortisol secretion using the lowest possible doses able to guarantee an adequate quality of life to patients 7.
• European Reference Networks on rare endocrine conditions aim to harmonize treatment and ensure access to specialized patient care for individual case-by-case treatment decisions 6.