Addison's disease

Addison's disease (also known as chronic adrenal insufficiency, or hypocortisolism) is a rare endocrine disorder, first described by British physician Thomas Addison. It is estimated that it affects about 1 to 2 in 100,000 people. It occurs when the adrenal glands, seated above the kidneys, fail to produce enough of the hormone cortisol and, sometimes, the hormone aldosterone. Addison's disease refers specifically to primary adrenal insufficiency, in which the adrenal glands themselves malfunction; secondary adrenal insufficiency occurs when the pituitary gland does not produce enough adrenocorticotropic hormone (ACTH) to adequately stimulate the adrenal glands. A famous sufferer of Addison's Disease was President John F. Kennedy. Jane Austen is thought to have been another.

Pathophysiology

Eighty to ninety percent of cases of Addison's disease are said to be due to autoantibodies directed against adrenal cells containing 21-hydroxylase, an enzyme involved in the production of cortisol and aldosterone. The remainder of cases are due to tuberculosis, HIV, sarcoidosis, amyloidosis, hemochromatosis, metastatic cancer to the adrenal glands, adrenal haemorrhage and congenital adrenal hyperplasia. Addison's disease can be an expression of an autoimmune polyendocrine syndrome when autoimmune reactions against other organs are also present. In APS type 1, 70% suffer from Addison's disease, while in type 2, 100% do. Through these syndromes, Addison's is associated with hypothyroidism, diabetes mellitus (type 1),vitiligo, alopecia and celiac disease.

Related Topics:
Tuberculosis - HIV - Sarcoidosis - Amyloidosis - Hemochromatosis - Cancer - Congenital adrenal hyperplasia - Autoimmune polyendocrine syndrome - Autoimmune - Hypothyroidism - Diabetes mellitus - Vitiligo - Alopecia - Celiac disease

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