Tuberculosi s : most common cause worldwide, but rare in the US.Associated with other autoimmune endocrinopat hies (see “ Autoimmune polyglandular syndromes”).Most common cause in the US ( ∼ 80–90% of all cases of primary adrenal insufficiency ).

Primary adrenal insufficiency can be caused by abrupt destruction of the adrenal gland ( acute adrenal insufficiency e.g., due to massive adrenal hemorrhage) or by its gradual progressive destruction or atrophy ( chronic adrenal insufficiency e.g., due to autoimmune conditions, infection). Primary adrenal insufficiency ( Addison disease )

It can be primary, secondary, or tertiary. EtiologyĪdrenal insufficiency is a failure of the adrenal glands to produce adequate amounts of adrenocortical hormones. Recommendations in this article are consistent with the 2016 Endocrine Society clinical practice guidelines for the diagnosis and treatment of primary adrenal insufficiency. Adrenal crisis is life-threatening and should be treated immediately with high doses of hydrocortisone and intravenous fluids. The dose of glucocorticoids should be increased during periods of stress (e.g., surgery, trauma, infections) in order to prevent adrenal crisis, which is a severe, acute type of adrenal insufficiency that manifests with shock, fever, impaired consciousness, and severe abdominal pain. Glucocorticoid replacement therapy with hydrocortisone is required for all forms of adrenal insufficiency. Serum cortisol levels that remain low even after the administration of exogenous ACTH ( ACTH stimulation test) confirm the diagnosis of primary adrenal insufficiency. Patients with primary adrenal insufficiency tend to additionally develop hyperpigmentation of the skin, mild hyperkalemia, and metabolic acidosis. There may also be a loss of libido as a result of hypoandrogenism. Patients with long-standing adrenal insufficiency can present with postural hypotension, nausea, vomiting, weight loss, anorexia, lethargy, depression, and/or chronic hyponatremia. Decreased levels of ACTH or CRH are seen following sudden cessation of prolonged glucocorticoid therapy and in pituitary/ hypothalamic diseases.

Secondary adrenal insufficiency is the result of decreased production of ACTH ( adrenocorticotropic hormone) and tertiary adrenal insufficiency is the result of decreased production of CRH ( corticotropin-releasing hormone) by the hypothalamus. The most frequent cause of primary adrenal insufficiency in the US by far is autoimmune adrenalitis, which can occur sporadically or as a manifestation of polyglandular autoimmune syndromes.

Primary adrenal insufficiency ( Addison disease) is caused by a disorder of the adrenal glands. Adrenal insufficiency is the decreased production of adrenocortical hormones ( glucocorticoids, mineralocorticoids, and adrenal androgens) and is classified as primary, secondary, or tertiary.

Adrenocortical insufficiency