Addison's disease is caused by deficient production of hormones by the
adrenal glands. In most cases this is caused by an autoimmune response that
destroys adrenal gland tissue. The condition occurs in about 1 in 100,000
people, at any age, at equal rates in men and women. Symptoms usually
begin gradually and include muscle weakness, fatigue, weight loss, low
blood pressure, and darkening of the skin.
Primary adrenal insufficiency in the pediatric population (0–18 years) is
most commonly attributed to congenital adrenal hyperplasia which occurs in
about 1 in 15,000 births, followed by Addison’s disease, with a likely
autoimmune etiology. Secondary forms often result from tuberculosis,
cancer, or surgery, in which the adrenal glands are destroyed or damaged. Of
patients with adrenocortical insufficiency, 33% also have diabetes. With
type 1 diabetes, the expression of organ-specific autoantibodies is very high
and this suggests a need for careful screening in both of these conditions.
There are two adrenal glands, located one above each kidney. The adrenal glands are really two endocrine (ductless or hormone producing) glands in one. The inner part of the adrenal gland produces epinephrine. The absence of the adrenal medulla and epinephrine does not cause disease. The outer portion of the adrenal gland, the cortex, is more critical. The adrenal cortex makes two important steroid hormones, cortisol and aldosterone.
Cortisol mobilizes nutrients, modifies the body's response to inflammation,
stimulates the liver to raise the blood sugar, and also helps to control the
amount of water in the body.
Aldosterone regulates salt and water levels which affects blood volume and
blood pressure. Cortisol production is regulated by another hormone,
adrenocorticotrophic hormone (ACTH), made in the pituitary gland which is
located just below the brain.
Classical Addison's disease results from a loss of both cortisol and
aldosterone secretion due to the near total or total destruction of both adrenal
glands. This condition is also called primary adrenal insufficiency. It affects
1 in 100,000 people. An Addison’s crisis can be precipitated by acute
infection, trauma, surgery, or excessive body salt loss. Adrenalectomy may
require steoroid replacement therapy, a 2 g sodium diet, and a control of
carbohydrate to prevent hyperglycemia. A normal lifespan is possible if
medication is taken as required.
If ACTH is deficient, there will not be enough cortisol produced, although
aldosterone may remain adequate. This is secondary adrenal insufficiency,
which is distinctly different, but similar to Addison's disease, since both
include a loss of cortisol secretion. This form is much more common. A
temporary form of secondary adrenal insufficiency may occur when a person
who has been receiving a glucocorticoid hormone such as prednisone for a
long time abruptly stops or interrupts taking the medication. Glucocorticoid
hormones, which are often used to treat inflammatory illnesses like
rheumatoid arthritis, asthma, or ulcerative colitis, block the release of both
corticotrophin-releasing hormone (CRH) and ACTH. Normally CRH
instructs the pituitary gland to release ACTH. If CRH levels drop, the
pituitary is not stimulated to release ACTH and the adrenals then fail to
secrete sufficient levels of cortisol. Hypoglycemia, anxiety, nausea, and
palpitations can occur.
In primary adrenal insufficiency, known as polyendocrine deficiency
syndrome, type-I occurs in children with under active parathyroid glands,
pernicious anemia, chronicCandida infections, chronic active hepatitis, and
slow sexual development. Autoimmune thyroid diseases are often associated
with type-I diabetes mellitus and Addison’s disease. Type-II primary adrenal
insufficiency affects young adults and presents with under active thyroid
gland, slow sexual development, diabetes, vitiligo, and loss of skin
pigmentation. Primary adrenal insufficiency causes abdominal pain,
vomitting, weakness, fatigue, weight loss, dehydration, nausea, diarrhea,
hyperpigmented skin, hypotension, low serum sodium, high serum
potassium, and low corticol steroid levels. Salt cravings can occur.
A medical history of the symptoms mentioned above, especially
hyperpigmentation of the skin or gums, is often enough to raise a strong
suspicion, prompting the appropriate tests. Quite often, however, the first
clue is from the abnormal results of routine tests done in a hospital or
doctor's office. These may include an elevated blood level of potassium, a
low blood level of sodium, a shift in the ratio of certain white blood cells, or
surprising changes on an EKG or chest X-ray that are caused by high
potassium or low blood volume. Other causes for these changes, particularly
from medications, must be considered first.
A definitive diagnosis of Addison's disease requires that definitive tests be
carried out. These tests measure the amount of cortisol and aldosterone in
the blood and urine, and document a lack of the normal increase in the levels
of these two hormones after administration of ACTH given by injection. An
elevated blood level of ACTH should also be found.
If the patient is very sick and Addison's disease is suspected, treatment can
be initiated while the diagnostic tests are being done. Once the diagnosis of
Addison's disease is established, an effort should be made to find the cause
by checking for tuberculosis and other infections through skin tests and X-
rays. Tuberculosis (TB), an infection which can destroy the adrenal glands,
accounts for about 20% of cases of primary adrenal insufficiency in
developed countries. When adrenal insufficiency was first identified by Dr.
Thomas Addison in 1849, TB was found at autopsy in 70 to 90% of cases.
As the treatment for TB improved, however, the incidence of adrenal
insufficiency due to TB of the adrenal glands has greatly decreased.
Antibodies to adrenal tissue, especially to 21OH can now be measured, and
are specific to autoimmune Addison’s.
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