Addison Disease (cont.)
What causes Addison's disease?
Failure to produce adequate levels of cortisol can occur for different reasons. The problem may be due to a disorder of the adrenal glands themselves (primary adrenal insufficiency) or to inadequate secretion of ACTH by the pituitary gland (secondary adrenal insufficiency).
Primary adrenal insufficiency
Addison's disease affects about 1 in 100,000 people. Most cases are caused by the gradual destruction of the adrenal cortex, the outer layer of the adrenal glands, by the body's own immune system. About 70 percent of reported cases of Addison's disease are caused by autoimmune disorders, in which the immune system makes antibodies that attack the body's own tissues or organs and slowly destroy them. Adrenal insufficiency occurs when at least 90 percent of the adrenal cortex has been destroyed. As a result, often both glucocorticoid (cortisol) and mineralocorticoid (aldosterone) hormones are lacking. Sometimes only the adrenal gland is affected, as in idiopathic adrenal insufficiency; sometimes other glands also are affected, as in the polyendocrine deficiency syndrome.
Polyendocrine deficiency syndrome
The polyendocrine deficiency syndrome is classified into two separate forms, referred to as type I and type II.
Type I occurs in children, and adrenal insufficiency may be accompanied by:
- underactive parathyroid glands
- slow sexual development
- pernicious anemia
- chronic candida infections
- chronic active hepatitis
- hair loss (in very rare cases)
Type II, often called Schmidt's syndrome, usually afflicts young adults.
Features of type II may include:
- an underactive thyroid gland
- slow sexual development
- diabetes
- vitiligo
- loss of pigment on areas of the skin
Scientists think that the polyendocrine deficiency syndrome is inherited
because frequently more than one family member tends to have one or more
endocrine deficiencies.
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