Addison's Disease

Addison's disease is a chronic condition involving metabolic dysfunction during which adrenal glands cannot produce enough of a particular hormone, which can, in turn, lead to fatigue, low blood pressure, loss of appetite, and the darkening of the pigmentation of the skin. This is caused by atrophy of the adrenal cortex.

Although the condition has been known for many years, it was the English physician Thomas Addison who managed to correlate a set of disease symptoms, and along with another physician, Richard Bright, at Guy's Hospital, London, wrote Elements of the Practice of Medicine (1839), and then wrote On the Constitutional and Local Effects of Disease of the Su-pra-Renal Capsules (1855). His book, An Essay on the Operation of Poisonous Agents upon the Living Body (1829), coauthored with John Morgan, was the first English book on the condition, which was named Addison's disease after him, with Addison's (pernicious) anemia also named after him.

The traditional development of Addison's disease is for it to get progressively worse over a number of months. Gradually, the patient encounters a loss of appetite, leading to weight loss, muscle weakness, and chronic fatigue as his/her condition deteriorates. This is often accompanied by nausea, diarrhea, or vomiting, with areas of hyperpigmentation—a darkening of skin color—and often depression and/or increased irritability. There can also be a craving for salt or salty food, with many women experiencing irregular menstrual periods, or periods that stop entirely.

Medical doctors examining patients for Addison's disease usually look for signs such as hypotension (low blood pressure), which gets worse when a person is standing; an increasing numbness in the extremities; decreased blood volume; dehydration; tetany, particularly after drinking milk; tremors; and restlessness. Often patients, especially children suffering from Addison's disease, will be found to have extremely low blood sugar levels. Diagnosis to confirm Addison's disease takes place when it has been shown that patients have adrenal hormone levels far lower than they should be, even after the stimulation of them with synthetic pituitary hormone.

The most common cause of Addison's disease in developed countries tends to be the autoimmune destruction of the adrenal cortex, which in turn leads to a proclivity to develop the adrenal autoimmune attack. Although this can happen in people without a family history of Addison's disease, for many people, the disease is inherited. A large genetic component has been found not only in humans, but also in some dog breeds, such as standard poodles and boxers. It is now felt that between 80 and 90 percent of all humans suffering from Addison's disease have it because their autoantibodies are directed against adrenal cells that contain 21-nydroxylase, the enzyme involved in the production of cortisol and aldosterone. Other cases can come from patients who have also been suffering from tuberculosis, HIV, sarcoidosis, amyloidosis, or metastic cancer.

The development of cortisone by the American biochemist Edward C. Kendall during the period leading up to its first introduction in 1948 has massively changed the manner of treating sufferers of Addison's disease. Kendall, along with fellow American Philip S. Hench and Swiss chemist and researcher Tadeus Reichstein, won the Nobel Prize for Physiology or Medicine in 1950. The methods of treating Addison's disease vary considerably, with the crux of the treatment being the attempt to replace the missing cortisol, and if necessary using fludrocortisones to replace the missing aldosterone. If a person has an acute attack, known as an Addisonian crisis, it is often treated with intravenous injections of cortisone (or cortisol), a saline solution, and/or glucose. The hormone replacement therapy using treatment with cortisone has to continue for life, often only controlling the symptoms of the disease rather than totally treating it.

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