From the Guidelines
Treatment for Addison's disease primarily involves hormone replacement therapy with oral hydrocortisone and fludrocortisone to replace cortisol and aldosterone, respectively, and patients must take these medications consistently for life to prevent life-threatening adrenal crisis. The standard treatment includes oral hydrocortisone (typically 15-25 mg daily, divided into 2-3 doses with the largest dose in the morning) to replace cortisol, and fludrocortisone (usually 0.05-0.2 mg once daily) to replace aldosterone 1. Patients must take these medications consistently for life, as stopping them can lead to life-threatening adrenal crisis. During times of illness, injury, or significant stress, patients need to increase their hydrocortisone dose (typically doubling or tripling it) to mimic the body's natural stress response, and the specific dosage adjustments are outlined in guidelines such as those provided by the UK Addison’s disease self-help group 1.
Some key points to consider in the management of Addison's disease include:
- The importance of increasing steroid doses during surgery and medical procedures according to the degree of stress induced, with specific recommendations provided in guidelines such as Table 6 from the UK Addison’s disease self-help group 1
- The need for patients to carry an emergency injection kit containing hydrocortisone for severe situations and wear a medical alert bracelet
- The importance of regular monitoring with blood tests and clinical evaluations to adjust medication doses as needed, with clinical assessment being the primary method of monitoring glucocorticoid replacement 1
- The potential for concomitant administration of several drugs to affect hydrocortisone efficacy, with CYP3A4 being the key drug metabolizing enzyme affecting hydrocortisone clearance 1
Overall, the goal of treatment for Addison's disease is to restore the hormonal balance that the adrenal glands can no longer maintain naturally, allowing patients to lead normal lives when properly managed, and regular monitoring and adjustment of medication doses is essential to achieve this goal.
From the FDA Drug Label
DOSAGE & ADMINISTRATION Dosage depends on the severity of the disease and the response of the patient. ... 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.
The treatment for Addison's disease is substitution therapy with a combination of fludrocortisone acetate and a glucocorticoid such as hydrocortisone or cortisone 2. The usual dose of fludrocortisone acetate is 0.1 mg daily, although dosage may vary from 0.1 mg three times a week to 0.2 mg daily 2.
- Fludrocortisone acetate is used as partial replacement therapy for primary and secondary adrenocortical insufficiency in Addison’s disease 2.
- Glucocorticoids such as hydrocortisone (10 mg to 30 mg daily) or cortisone (10 mg to 37.5 mg daily) are administered in conjunction with fludrocortisone acetate 2.
From the Research
Treatment Overview
The treatment for Addison's disease typically involves replacing the deficient hormones with medication.
- The hormone deficiencies in Addison's disease are conventionally treated with oral glucocorticoid and mineralocorticoid replacement 3, 4, 5.
- 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, 5.
Emerging Therapies
New treatment modalities have been developed to improve the management of Addison's disease.
- Timed-release hydrocortisone tablets and continuous subcutaneous hydrocortisone infusion are promising new treatment modalities 3, 4.
- Modified-released hydrocortisone and continuous subcutaneous hydrocortisone infusion offer the potential for closer simulation of the physiological serum cortisol rhythm 4.
- Studies have also looked at modifying the natural history of adrenal failure using adrenocorticotropic hormone (ACTH) stimulation and immunomodulatory therapies 4.
Monitoring and Adjustment
Monitoring and adjusting the treatment regimen is crucial to optimize the management of Addison's disease.
- Salivary cortisol day curves (SCDC) can be used to individualize glucocorticoid replacement therapy (GRT) and approach normal cortisol levels as closely as possible 6.
- Adjusting GRT based on SCDC can reduce overreplacement, especially in the evening, and lead to a reduction of sleep disturbances and fatigue in patients with Addison's disease 6.