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$Unique_ID{BRK03427}
$Pretitle{}
$Title{Addison's Disease}
$Subject{Addison's Disease Adrenal Hypoplasia Adrenocortical Hypofunction
Adrenocortical Insufficiency Chronic Adrenocortical Insufficiency Primary
Adrenal Insufficiency Primary Failure Adrenocortical Insufficiency
Adrenoleukodystrophy Amyloidosis ACTH Deficiency Cushing's Syndrome Schmidt
Syndrome Adrenal Hyperplasia, Congenital (CAH)}
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Copyright (C) 1985, 1986, 1988, 1991, 1993 National Organization for Rare
Disorders, Inc.

46:
Addison's Disease

** IMPORTANT **
It is possible that the main title of the article (Addison's Disease) is
not the name you expected.  Please check the SYNONYMS listing to find the
alternate name and disorder subdivisions covered by this article.

Synonyms

     Adrenal Hypoplasia
     Adrenocortical Hypofunction
     Adrenocortical Insufficiency
     Chronic Adrenocortical Insufficiency
     Primary Adrenal Insufficiency
     Primary Failure Adrenocortical Insufficiency

Information on the following diseases can be found in the Related
Disorders section of this report:

     Adrenoleukodystrophy
     Amyloidosis
     ACTH Deficiency
     Cushing's Syndrome
     Schmidt Syndrome
     Adrenal Hyperplasia, Congenital (CAH)

General Discussion

** REMINDER **
The Information contained in the Rare Disease Database is provided for
educational purposes only.  It should not be used for diagnostic or treatment
purposes.  If you wish to obtain more information about this disorder, please
contact your personal physician and/or the agencies listed in the "Resources"
section of this report.


Addison's Disease is a rare disorder characterized by chronic, usually
progressive, insufficient functioning of the outer layer of the adrenal
glands (adrenal cortex).  Deficiencies of cortisol and aldosterone (hormones
manufactured in the adrenals) result in low levels of sodium and chloride in
the blood and body tissues, and high levels of potassium (electrolyte
imbalance) in the body.  Increased excretion of water and low blood pressure
(hypotension) can lead to dehydration (extremely low levels of water in the
body).  Major symptoms of Addison's Disease include fatigue, gastrointestinal
discomfort, and changes in skin color (pigmentation).

Symptoms

The early symptoms of Addison's Disease may include:  weakness, fatigue, loss
of appetite (anorexia), increased water excretion, low blood pressure
(hypotension), and darkened color of scars, skin folds, and/or mucous
membranes (hyperpigmentation).  Black freckles may appear on the head and
shoulders.

Later symptoms of Addison's Disease may include:  nausea, dehydration as
a result of vomiting and diarrhea, dizziness, and/or the inability to
withstand exposure to cold temperatures.  Other symptoms may also include:
episodes of unconsciousness and/or fainting (syncopal attacks), general
indifference to the events of daily living (apathy), mental confusion, fever,
abdominal pain, and/or low blood sugar (hypoglycemia).

An acute life-threatening state of extreme insufficiency of
adrenocortical hormones (adrenal or "Addisonian" crisis) may occur in people
with Addison's Disease.  This crisis may begin as a sudden loss of strength,
severe pain in the abdomen, lower back, and/or kidney failure.

Causes

The exact cause of Addison's Disease and the associated loss of function of
the adrenal cortex is not known.  Approximately 75 percent of cases of
Addison's Disease are thought to be autoimmune related.  Autoimmune disorders
occur when the body's natural immune defenses (antibodies, lymphocytes,
etc.), against invading organisms mistakenly attack perfectly healthy tissue.

Other cases of Addison's Disease may be caused by the partial destruction
of the adrenal cortex due to other disorders including tuberculosis, the
growth of a tumor, and/or the abnormal accumulation of a fatty-like substance
within the adrenals (Amyloidosis).   Acute infection, trauma, surgery, and/or
sodium loss caused by heavy sweating can trigger an adrenal crisis.  (For
more information on these disorders, choose "Tuberculosis" and/or
"Amyloidosis" as your search terms in the Rare Disease Database.)

Affected Population

Addison's Disease affects males and females in equal numbers and may occur at
any age.  Approximately 4 in 100,000 people in the United States are affected
with Addison's Disease.

Related Disorders

Symptoms of the following disorders can be similar to those of Addison's
Disease.  Comparisons may be useful for a differential diagnosis:

Adrenoleukodystrophy is a rare inherited metabolic disorder characterized
by abnormally high levels of very long chain fatty acids (VLCFA) in the blood
plasma and tissues of the body.  This results in the progressive loss of the
fatty covering (myelin sheath) on nerve fibers within the brain (cerebral
demyelination) and the degeneration of the adrenal glands (adrenal atrophy).
Symptoms of Adrenoleukodystrophy may include:  progressive spastic partial
paralysis (paraparesis), an impaired ability to coordinate muscle movement
(ataxia), and/or muscular rigidity (hypertonia).  The symptoms of decreased
adrenal gland function associated with Adrenoleukodystrophy may include:  low
blood pressure (hypotension), generalized weakness, fatigue, excessive loss
of water from body tissues (dehydration), weight loss, an abnormally small
heart (microcardia), an increase of color (pigmentation) in the skin, and/or
a decrease in the secretion of adrenal hormones.  (For more information on
this disorder, choose "Adrenoleukodystrophy" as your search term in the Rare
Disease Database.)

Amyloidosis is the term applied to a group of metabolic disorders in
which amyloid (a fibrous protein) accumulates in tissues of the body.  The
excessive accumulation of amyloid causes the affected organ to malfunction;
accumulation may be localized, general, or systemic.  Secondary Amyloidosis
commonly impairs the function of the kidneys, liver, spleen, and/or adrenal
glands.  The symptoms of Amyloidosis are varied and relate to the organs that
have an amyloid buildup.  Generally the involved organs become enlarged and
have a firm, waxy appearance.  (For more information on this disorder, choose
"Amyloidosis" as your search term in the Rare Disease Database.)

ACTH Deficiency is a rare endocrine disorder characterized by decreased
or absent adrenocorticotropic hormone (ACTH) which is normally produced in
the pituitary gland.  The adrenal glands produce abnormally low levels of
cortisol and steroid hormones (secondary adrenal insufficiency).  The
symptoms of this disorder may include:  weight loss, profound loss of appetite
(anorexia), muscle weakness, nausea, vomiting, and/or low blood pressure
(hypotension).  (For more information on this disorder, choose "ACTH
Deficiency" as your search term in the Rare Disease Database.)

Cushing's Syndrome is a rare disorder of the outer layers of the adrenal
glands (adrenal cortex) that is associated with many symptoms resulting from
excess amounts of corticosteroids produced by the adrenal cortex.  Typically
people with Cushing's Syndrome gain excessive weight; fat deposits result in
a rounded shape to the face, neck, and trunk of the body.  Typically the legs
and arms remain slender.  Other symptoms include:  redness to the face, thin
and fragile skin, slow healing, and/or reddish-blue stretch marks on the
arms, breasts, abdomen, and thighs.  Low blood sugar (hyperglycemia), severe
weakness and fatigue are common in people with Cushing's Syndrome.  (For more
information on this disorder, choose "Cushing" as your search term in the
Rare Disease Database.)

Schmidt Syndrome is a rare disorder characterized by multiple hormone
deficiencies, the presence of Addison's Disease, and decreased activity of
the thyroid gland.  Others glands that may demonstrate decreased levels of
hormone secretion include the parathyroids, testes or ovaries, and/or the
pancreas.  A Vitamin B12 deficiency may also occur.  Symptoms of Schmidt
Syndrome may include a general loss of energy, muscle weakness, anemia,
fatigue, shortness of breath, loss of hair (alopecia), and/or the appearance
of white spots on various parts of the body (vitiligo).  (For more
information on this disorder, choose "Schmidt" as your search term in the
Rare Disease Database.)

Congenital Adrenal Hyperplasia (CAH) is a group of rare endocrine
disorders resulting from a defect in the production of hormones in the
adrenal glands (adrenal corticosteroids).   The adrenal glands become
enlarged and produce abnormally excessive amounts of androgens resulting in
abnormalities of sexual development.   Low levels of hormones, particularly
cortisol, may cause a variety of metabolic problems and symptoms of Addison's
Disease.  Symptoms may include:  weakness, nausea, vomiting, anorexia,
irritability, depression, darkening of the skin, and/or low blood pressure.
(For more information on this disorder, choose "Congenital Adrenal
Hyperplasia" as your search term in the Rare Disease Database.)

Therapies:  Standard

The chronic adrenal insufficiency that characterizes Addison's Disease is
treated with replacement therapy that consists of cortisone and
fludrocortisone; these replacement hormones are taken with meals.  The dosage
of these drugs should be increased during infection, trauma, surgery, and
other stress to prevent an acute adrenal crisis.

An adrenal crisis demands immediate intravenous administration of high-
dose hydrocortisone succinate or phosphate and fluid and electrolyte
replacement; a short-term course of other drugs called vasopressors may be
needed to maintain blood pressure.  Patients should carry a card or wear a
tag stating that they have Addison's disease.

Therapies:  Investigational

The National Institute of Diabetes, Digestive & Kidney Diseases (NIDDK) is
conducting a study on Addison's Disease.  For more information have your
physician contact:

     Joan Chamberlain, Deputy Director
     NIH/National Institute of Diabetes, Digestive & Kidney Diseases
     Building 31, Room 9A-04
     9000 Rockville Pike
     Bethesda, Maryland 20892

This disease entry is based upon medical information available through
March 1993.  Since NORD'S resources are limited, it is not possible to keep
every entry in the Rare Disease Database completely current and accurate.
Please check with the agencies listed in the Resource section for the most
current information about this disorder.

Resources

For more information on Addison's Disease, please contact:

     National Organization for Rare Disorders (NORD)
     P.O. Box 8923
     New Fairfield, CT  06812-1783
     [phone]

     The National Adrenal Diseases Foundation, Inc.
     500 Northern Blvd., Suite 200
     Great Neck, NY  11021
     [phone]

     National Digestive Diseases Information Clearinghouse
     P.O. Box NDDIC
     Bethesda, MD  20892
     [phone]

References

CECIL TEXTBOOK OF MEDICINE, 19th Ed.:  James B. Wyngaarden, and Lloyd H.
Smith, Jr., Editors; W.B. Saunders Co., 1990.  Pp. 1340, 1460-61.

TEXTBOOK OF ENDOCRINE PHYSIOLOGY, James E. Griffin, and Sergio R. Ojeda.,
Editors; Oxford University Press, 1988.  Pp. 258-9.

MENDELIAN INHERITANCE IN MAN, 10th Ed.:  Victor A. McKusick, Editor:
Johns Hopkins University Press, 1992.  P. 36.

DELAYED DIAGNOSIS OF ADDISON'S DISEASE. J.R. Paterson, et al.; Ann Clin
Biochem (July, 1990; 27(PT 4)).  Pp. 378-81.

SCHMIDT'S SYNDROME:  A RARE CAUSE OF PUBERTY MENORRHAGIA.  J.B. Sharma, et
al.; Int J Gynaecol (December, 1990; 33(4)).  Pp. 373-75.

HYPERKALAEMIC PERIODIC PARALYSIS:  A RARE PRESENTATION OF ADDISON'S
DISEASE.  J.M. Sowden, et al.; Postgrad Med J (April, 1989; 65(762)).  Pp.
238-40.