In the past, the most common cause of primary adrenal disease was tuberculosis. Now, most cases are designated as idiopathic and thought to represent an autoimmune disorder, often associated with Hashimoto thyroiditis and diabetes mellitus and rarely with other polyglandular autoimmune endocrine disorders. A less-frequent cause is a hereditary metabolic disease of the adrenals—in combination with a demyelinating disease of brain, spinal cord, and nerves and occurring predominantly in males (adrenoleukodystrophy; see Chap. 37). In primary adrenal disease, plasma concentrations of cortisol are low and the serum ACTH level rises as a consequence. Adrenal insufficiency of whatever cause is a life- threatening condition; there is always a danger of collapse and even death, particularly during periods of infection, surgery, and extreme injury. Lifelong replacement therapy is usually required with a glucocorticoid (cortisone, 25 to 50 mg, or prednisone, 7.5 to 15 mg daily) and a mineralocorticoid, such as fludrocortisone acetate (Florinef), 0.05 to 0.2 mg daily.
When adrenal insufficiency is secondary to disease of the pituitary, ACTH is low or absent and cortisol secretion is markedly reduced, but aldosterone levels are sustained. Hyperpigmentation is notably absent; it is the elevation of ACTH that causes melanoderma, such as occurs, for example, in patients subjected to bilateral adrenalectomy. Hypothalamic lesions, principally involving the paraventricular nuclei, may also cause adrenal insufficiency, but less frequently than do pituitary lesions.
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