What is the difference between primary adrenal insufficiency (Addison's disease) and primary adrenocortical insufficiency?

From the Guidelines

Primary adrenal insufficiency (Addison's disease) and primary endocortical adrenal insufficiency refer to the same condition, with no difference between these terms, as both describe a disorder where the adrenal glands fail to produce sufficient amounts of cortisol and often aldosterone due to damage to the adrenal cortex itself. This condition is typically treated with glucocorticoid replacement (usually hydrocortisone 15-25 mg daily in divided doses or prednisolone 3-5 mg daily) and mineralocorticoid replacement (fludrocortisone 0.05-0.2 mg daily) if aldosterone is deficient, as recommended by the most recent guidelines 1. Patients need to carry emergency medication and identification, increase medication during illness or stress, and have regular follow-up appointments to adjust dosing. The condition results from autoimmune destruction of the adrenal glands in about 80% of cases in developed countries, but can also be caused by infections (particularly tuberculosis), hemorrhage, metastatic cancer, or genetic disorders, as noted in a consensus statement on the diagnosis, treatment, and follow-up of patients with primary adrenal insufficiency 1. The term "endocortical" simply emphasizes that the problem affects the cortex (outer layer) of the adrenal gland rather than the medulla (inner portion).

Key Points

• Primary adrenal insufficiency and primary endocortical adrenal insufficiency are the same condition
• Treatment involves glucocorticoid and mineralocorticoid replacement
• Patients require regular follow-up and education on managing their condition
• The condition can result from various causes, including autoimmune destruction, infections, and genetic disorders

Management

• Glucocorticoid replacement: hydrocortisone 15-25 mg daily in divided doses or prednisolone 3-5 mg daily
• Mineralocorticoid replacement: fludrocortisone 0.05-0.2 mg daily if aldosterone is deficient
• Emergency medication and identification are essential
• Regular follow-up appointments are necessary to adjust dosing and monitor the patient's condition, as recommended by guidelines for the management of glucocorticoids during the peri-operative period for patients with adrenal insufficiency 1.

From the FDA Drug Label

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. 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 FDA drug label does not answer the question.

From the Research

Definition and Causes

• Primary adrenal insufficiency, also known as Addison's disease, is a rare endocrine disorder characterized by the insufficient production of hormones by the adrenal cortex 2, 3, 4, 5.
• The most common cause of primary adrenal insufficiency is autoimmune adrenalitis, which results from the destruction of the adrenal cortex 3.
• Other causes of primary adrenal insufficiency include infections, tumors, and genetic disorders 4.

Clinical Manifestations

• The clinical manifestations of primary adrenal insufficiency can be nonspecific and include fatigue, weight loss, hypotension, hyperpigmentation, and salt craving 2, 3, 4, 5.
• Patients with primary adrenal insufficiency may also experience anorexia, nausea, muscle and joint pain, and orthostatic hypotension 3.
• In acute adrenal crisis, patients may present with orthostatic hypotension, fever, and hypoglycemia 5.

Diagnosis

• The diagnosis of primary adrenal insufficiency is often delayed or mistaken for secondary adrenal insufficiency, which can have life-threatening consequences 6.
• The diagnostic test of choice is dynamic testing with synthetic adrenocorticotropin (ACTH) 4, 5.
• Measurement of basal plasma cortisol is an insensitive screening test, but elevated plasma levels of adrenocorticotropin and renin can confirm the diagnosis 5.
• Morning cortisol concentrations can be used to make a diagnosis, with values above 350 nmol/l considered to safely rule out Addison's disease 2.

Treatment

• Treatment of primary adrenal insufficiency requires replacement of mineralocorticoids and glucocorticoids 3, 4, 5.
• Patients with primary adrenal insufficiency require lifelong hormone supplementation and education in dose adaptation during physical and mental stress or emergency situations to prevent life-threatening adrenal crises 4.
• Patients should carry an emergency card and emergency kit with them at all times 4.