From the Guidelines
Adrenal insufficiency can be caused by several diseases, including primary adrenal insufficiency (Addison's disease), secondary adrenal insufficiency, and tertiary adrenal insufficiency, as well as congenital conditions like congenital adrenal hyperplasia and adrenoleukodystrophy. The causes of adrenal insufficiency can be categorized into primary, secondary, and tertiary, with primary adrenal insufficiency resulting from direct damage to the adrenal glands, commonly caused by autoimmune adrenalitis, tuberculosis, fungal infections, metastatic cancer, adrenal hemorrhage, and certain medications like ketoconazole and etomidate 1. Some of the key diseases that cause adrenal insufficiency include:
- Autoimmune adrenalitis
- Tuberculosis
- Fungal infections
- Metastatic cancer
- Adrenal hemorrhage
- Certain medications like ketoconazole and etomidate
- Congenital adrenal hyperplasia (particularly 21-hydroxylase deficiency)
- Adrenoleukodystrophy
- Pituitary tumors
- Pituitary surgery or radiation
- Hypopituitarism
- Prolonged corticosteroid therapy leading to HPA axis suppression
- Hypothalamic dysfunction affecting CRH production According to the most recent guidelines, patients with primary adrenal insufficiency should take 15–25 mg of hydrocortisone (HC) daily in split doses, and 50–200 µg fludrocortisone as a single daily dose 1. It is essential to note that adrenal crisis is a life-threatening emergency requiring immediate hydrocortisone injection (100 mg) and fluid resuscitation 1. In addition, patients with adrenal insufficiency should wear Medic Alert identification jewelry and carry a steroid/alert card, and receive sufficient education to manage daily medications and situations of minor to moderate concurrent illnesses 1. The prevalence of adrenal insufficiency in adult and pediatric populations is significant, with approximately 1 in 8–10,000 children affected by primary adrenal insufficiency, and 1 in 15,000 live births affected by congenital adrenal hyperplasia 1. Overall, the management of adrenal insufficiency requires a comprehensive approach, including hormone replacement therapy, patient education, and emergency preparedness.
From the FDA Drug Label
Primary or secondary adrenocortical insufficiency (hydrocortisone or cortisone is the first choice; synthetic analogs may be used in conjunction with mineralocorticoids where applicable; in infancy mineralocorticoid supplementation is of particular importance)
The diseases that cause adrenal insufficiency include:
- Primary adrenocortical insufficiency
- Secondary adrenocortical insufficiency 2
From the Research
Adrenal Insufficiency Causes and Related Diseases
Adrenal insufficiency is a condition where the adrenal glands do not produce adequate amounts of hormones, such as glucocorticoids and mineralocorticoids. This can be caused by primary adrenal insufficiency, where the adrenal gland fails, or secondary adrenal insufficiency, where there is a lack of stimulation from the pituitary gland or hypothalamus 3.
Diseases Related to Adrenal Insufficiency
Some of the diseases related to adrenal insufficiency include:
- Addison's disease, a primary adrenal insufficiency disorder 4
- Hypopituitarism, a secondary adrenal insufficiency disorder 4
- Cushing's syndrome, which can occur due to over-treatment with glucocorticoids 4
- Adrenal crisis, a life-threatening medical emergency that requires immediate treatment with fluids and corticosteroids 3, 5
Symptoms and Diagnosis
Adrenal insufficiency can cause non-specific symptoms, and early detection and testing are crucial to prevent adrenal crisis 3. Diagnosis can be made using a low baseline cortisol level, raised adrenocorticotropic hormone (ACTH), and confirmatory tests such as the cosyntopin stimulation test or insulin tolerance test 3.
Treatment and Management
Treatment for adrenal insufficiency typically involves glucocorticoid replacement therapy, which can be twice- or thrice-daily dosing with a glucocorticoid, such as oral hydrocortisone 4. However, modern hydrocortisone replacement regimens may be associated with an increased risk of adrenal crisis 5. Patients must be educated and empowered to take control of their own medical management to prevent adrenal crisis and other complications 3.