From the Guidelines
Adrenal axis insufficiency should be treated with hormone replacement therapy, typically involving glucocorticoids such as hydrocortisone at 15-25 mg daily, and mineralocorticoid replacement with fludrocortisone at 0.05-0.2 mg daily for primary adrenal insufficiency. The treatment approach is based on the most recent and highest quality study available, which emphasizes the importance of hormone replacement therapy in managing adrenal axis insufficiency 1.
Key Considerations
- Hydrocortisone is the preferred glucocorticoid for replacement therapy, given its ability to mimic the body's natural cortisol rhythm, with 2/3 of the dose typically administered in the morning and 1/3 in the early afternoon 1.
- Patients with primary adrenal insufficiency require both glucocorticoid and mineralocorticoid replacement, with fludrocortisone being the standard mineralocorticoid used 1.
- Stress dosing is crucial during illness, injury, or surgery, with doses typically increased to 2-3 times the normal maintenance dose 1.
- Regular monitoring of symptoms, blood pressure, and electrolytes is essential to adjust medication doses and prevent complications such as adrenal crisis 1.
Management and Follow-Up
- Patients should be educated on stress dosing, the use of emergency injectables, and the importance of wearing medical alert identification 1.
- Endocrinology consultation is recommended for all patients with adrenal axis insufficiency, particularly for planning before surgery or high-stress treatments 1.
- Follow-up appointments should be scheduled at least annually to assess the patient's health and well-being, with occasional monitoring for the development of new autoimmune disorders and complications of glucocorticoid therapy 1.
From the Research
Adrenal Axis Insufficiency
- Adrenal insufficiency (AI) is a life-threatening condition that requires lifelong glucocorticoid substitution therapy and stress adaptation to prevent adrenal crises 2.
- The current standard treatment regimen for AI involves twice- or thrice-daily dosing with a glucocorticoid, most commonly oral hydrocortisone 3.
- However, data from the past 10-15 years have shown that morbidity remains high and life expectancy is reduced in patients with AI, despite conventional glucocorticoid replacement therapy 3.
- New treatment modalities, such as modified-released hydrocortisone and continuous subcutaneous hydrocortisone infusion, have been developed to offer closer simulation of the physiological serum cortisol rhythm 4.
Causes and Management
- Primary adrenal insufficiency (Addison's disease) refers to glucocorticoid deficiency resulting from adrenal gland disease, while central adrenal insufficiency is caused by disorders of the pituitary gland (secondary) or the hypothalamus (tertiary) 5.
- Long-term hypothalamic-pituitary-adrenal (HPA) axis suppression is commonly seen after chronic glucocorticoid therapy (iatrogenic), and transient HPA axis suppression is increasingly being reported in hospitalized patients with acute illness (relative adrenal insufficiency) 5.
- Treatment of adrenal insufficiency consists of immediate parenteral hydrocortisone and subsequent continuous infusion until the underlying precipitating cause is cured and/or when regular supplementation can be continued 6.
Therapy Options
- Various glucocorticoid formulations are available, including short-acting, intermediate, long-acting, and novel modified-release hydrocortisone, as well as subcutaneous formulations 2.
- 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 2.
- Educational programs and emergency cards are necessary to train patients, relatives, and spouses in prevention and early recognition of adrenal crisis 6.