Addison Disease

Excerpt

Addison disease, also known as autoimmune adrenalitis, is an acquired primary adrenal insufficiency. Primary adrenal insufficiency is termed Addison disease when an autoimmune process causes the condition and is a rare but potentially life-threatening emergency condition. Addison disease results from the destruction of the bilateral adrenal cortex, leading to decreased adrenocortical hormones, including cortisol, aldosterone, and androgens. Addison disease's insidious course of action usually presents with glucocorticoid deficiency followed by mineralocorticoid. However, the condition can also present acutely, often triggered by intercurrent illness. The presentation of adrenal insufficiency depends on the rate and extent of adrenal function involvement. The most common cause of primary adrenal insufficiency is Addison disease, associated with increased levels of 21-hydroxylase antibodies.

Addison disease usually manifests as an insidious and gradual onset of nonspecific symptoms, often resulting in a delayed diagnosis. The symptoms may worsen over a period, which makes early recognition difficult. A high clinical suspicion should be maintained to avoid misdiagnosis. In many cases, the diagnosis is made only after the patient presents with an acute adrenal crisis manifesting with hypotension, hyponatremia, hyperkalemia, and hypoglycemia. The diagnosis is established by demonstrating low cortisol and aldosterone levels, high renin levels, and a blunt cortisol response with ACTH stimulation. Addison crisis is a severe endocrine emergency; immediate recognition and treatment are required. For stabilized patients diagnosed with Addison disease, life-long treatment with hormonal replacement is needed. Maintenance therapy aims to provide a replacement to maintain a physiologic glucocorticoid and mineralocorticoid level.