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$Unique_ID{BRK04329}
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$Title{Whipple's Disease}
$Subject{Whipple's Disease Intestinal Lipodystrophy Secondary Non-tropical
Sprue Intestinal Lipophagic Granulomatosis Crohn's Disease Ulcerative Colitis
Primary Sclerosing Cholangitis Chronic Erosive Gastritis Glucose-Galactose
Malabsorption Irritable Bowel Syndrome Intestinal Pseudoobstruction Giant
Hypertrophic Gastritis AIDS }
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Copyright (C) 1986, 1990, 1993 National Organization for Rare Disorders,
Inc.

90:
Whipple's Disease

** IMPORTANT **
It is possible that the main title of the article (Whipple'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

     Intestinal Lipodystrophy
     Secondary Non-tropical Sprue
     Intestinal Lipophagic Granulomatosis

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

     Crohn's Disease
     Ulcerative Colitis
     Primary Sclerosing Cholangitis
     Chronic Erosive Gastritis
     Glucose-Galactose Malabsorption
     Irritable Bowel Syndrome
     Intestinal Pseudoobstruction
     Giant Hypertrophic Gastritis
     AIDS

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.


Whipple's Disease is a rare infectious disease that causes an abnormality
in the metabolism and/or usage of fats (lipodystrophy) in the small intestine
(localized) characterized by impairment of the ability to properly absorb
nutrients (malabsorption), anemia, and joint pain.  This disorder may also
affect other organs of the body including the heart, lungs, brain, and eyes.

Symptoms

The major symptoms of Whipple's Disease include abdominal pain after eating,
joint pain, bouts of diarrhea, cough, chest pain, general weakness, and night
sweats.  Typically there is fat present in the stool (steatorrhea).  Weight
loss may occur because of a profound lack of appetite (anorexia).  Anemia may
result due to insufficient levels of iron.

Other symptoms of Whipple's Disease may include:  abnormally enlarged
lymph nodes that are firm but usually not tender, an abnormally enlarged
spleen (splenomegaly), increased color (pigmentation) of the skin, a decrease
in blood pressure (hypotension), and abnormally high fevers that come and go.
Some people with this disorder may experience a decrease in intellectual
abilities, and an impairment of memory, judgment, and/or abstract thought.
Occasionally the loss of intellectual skills progresses to dementia.  Eye
movements may be impaired and uncontrolled muscular movements (myoclonus) may
occur when Whipple's Disease has affected the brain or central nervous
system.  (For more information on this disorder, choose "Myoclonus" as your
search term in the Rare Disease Database.)

The central nervous system is affected in the later stages of untreated
Whipple's Disease.  Symptoms of neurological involvement may include hearing
loss, persistent ringing in the ears (tinnitus), and impairment of vision.
(For more information on this disorder, choose "Tinnitus" as your search term
in the Rare Disease Database.)  In rare cases of this disorder, the heart may
be affected resulting in congestive heart failure and/or inflammation of the
membranes that surround the heart (pericarditis).

If Whipple's Disease remains untreated and malabsorption from the small
intestine becomes worse, the person may have low levels of circulating
calcium and magnesium in the blood (hypokalemia and hypomagnesemia) resulting
in muscle cramps, convulsions, and twitching (tetany).  Damage to the nerves,
especially those of the arms and legs (peripheral neuropathy) may also occur.
(For more information on these disorders, choose "Neuropathy, Peripheral" as
your search term in the Rare Disease Database.)

Whipple's Disease may be diagnosed by ultrasound tests and CT scan that
may reveal abnormally enlarged lymph nodes (lymphadenopathy) and/or a
thickening of the lining of the small intestine.  Biopsy samples of the small
intestine reveal the presence of the bacteria that causes this disorder.
Without proper antibiotic treatment, Whipple's Disease may result in life-
threatening complications.

Causes

Whipple's Disease is caused by a "rod-shaped" bacterium called Tropheryma
whippelii.

Affected Population

Whipple's Disease is a rare disorder that affects more males than females in
a ratio of 8 males to 1 female.  The symptoms of this disorder typically
begin between the ages of thirty and sixty years.  Most of the cases of
Whipple's Disease have been diagnosed in Americans of European descent,
although cases have been reported among American Indians and Americans of
African descent.

Related Disorders

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

Crohn's Disease is an inflammatory bowel disease characterized by severe,
chronic inflammation of the intestinal wall or any portion of the
gastrointestinal tract.  Symptoms may include vomiting, fever, night sweats,
loss of appetite, general weakness, and waves of abdominal pain and
discomfort.  Diarrhea and bleeding from the rectum are common in people who
have Crohn's Disease.  Weight loss is also common.  The symptoms of this
disorder can be difficult to manage and diagnosis is often delayed.  (For
information on this disorder, choose "Crohn's Disease" as your search term in
the Rare Disease Database.)

Ulcerative Colitis is an acute inflammatory bowel disease characterized
by diarrhea and blood in the stools because of multiple, irregular
ulcerations of the bowel.  The initial symptoms of this disorder may include
a general feeling of weakness (malaise) and fatigue.  There may be abdominal
discomfort, along with a change in the frequency and consistency of stools.
Other symptoms may include abdominal pain, cramping, and urgency (tenesmus).
Weight loss and a decrease in appetite are also associated with Ulcerative
Colitis.  (For more information on this disorder, choose "Ulcerative Colitis"
as your search term in the Rare Disease Database.)

Primary Sclerosing Cholangitis is a rare collagen disorder involving
inflammation and blockage of the bile duct, liver ducts, and gallbladder.
Symptoms of this disorder include abdominal pain, loss of appetite, nausea,
vomiting, and/or weight loss.  Later symptoms may include a yellow
discoloration to the skin (jaundice), fever, chills, and/or itching of the
skin.  Bacterial infections may be associated with bile duct blockages of
Primary Sclerosing Cholangitis.  (For more information on this disorder,
choose "Primary Sclerosing Cholangitis," as your search term in the Rare
Disease Database.)

Chronic Erosive Gastritis is an inflammatory digestive disorder
characterized by multiple lesions in the mucus lining of the stomach.
Symptoms of this disorder may include burning or a heavy feeling in the
stomach, mild nausea, vomiting, loss of appetite and general weakness.  In
severe cases of Chronic Erosive Gastritis there may be bleeding from the
stomach that can result in anemia.  (For more information on this disorder,
choose "Chronic Erosive Gastritis" as your search term in the Rare Disease
Database.)

Glucose-Galactose Malabsorption (carbohydrate intolerance) is a rare
inherited disorder characterized by the inability of the small intestine to
transport and absorb glucose and galactose.  The symptoms may include
diarrhea, bloating, dehydration, profound loss of appetite, and vomiting.
Other symptoms may include abdominal cramps, and rumbling sounds caused by
gas in the intestine (borborygmi), and/or excessive urination.  (For more
information on this disorder, choose "Glucose-Galactose Malabsorption" as
your search term in the Rare Disease Database.)

Irritable Bowel Syndrome, also known as Spastic Colon, is a common
digestive disorder that involves both the small intestine and the large
bowel.  This disorder is characterized by abdominal pain, constipation,
bloating, nausea, headache, and/or diarrhea.  The spastic colon type of this
syndrome is characterized by variable bowel movements and abdominal pain that
is associated with periodic constipation or diarrhea.  Those patients with
Irritable Bowel Syndrome who have painless diarrhea may experience an urgent
need to defecate upon arising.  (For more information on this disorder,
choose "Irritable Bowel Syndrome" as your search term in the Rare Disease
Database.)

Intestinal Pseudoobstruction is a gastrointestinal disorder characterized
by a lack of motility of the intestine.  This condition resembles a true
obstruction although there is no evidence of any physical obstruction.
Symptoms may include constipation, colicky pain, vomiting, and weight loss.
Intestinal Pseudoobstruction may also affect speech, muscle activity, and the
nervous system.  (For more information on this disorder, choose "Intestinal
Pseudoobstruction, Intestinal" as your search term in the Rare Disease
Database.)

Giant Hypertrophic Gastritis is a chronic disorder characterized by the
presence of large, coiled ridges or folds, in the inner wall of the stomach.
Symptoms include abdominal pain or discomfort and tenderness in the upper
middle region of the abdomen.  Other symptoms may include a profound loss of
appetite, nausea, vomiting, and diarrhea.  (For more information on this
disorder, choose "Giant Hypertrophic Gastritis" as your search term in the
Rare Disease Database.)

Acquired Immune Deficiency Syndrome (AIDS) is an immunosuppressive
disorder caused by infection with the human immunodeficiency virus (HIV).
The immune deficiency is a result of a viral infection and the destruction of
specific T cells.  Initially HIV infection is characterized by a period
without symptoms.  This may be followed by the development of swollen lymph
nodes (lymphadenopathy).  Eventually most people with Acquired Immune
Deficiency Syndrome experience a progression of symptoms that occur as a
result of a compromised immune system.  When a person with AIDS has an
intestinal infection with Mycobacterium avium intracellulare, the symptoms
may be confused with those of Whipple's Disease.  (For more information on
this disorder, choose "AIDS" as your search term in the Rare Disease
Database.)

Therapies:  Standard

Treatment of Whipple's Disease includes the use of antibiotics.  Many
different types have been helpful; e.g., tetracycline, chlortetracycline,
sulfasalizine, ampicillin, trimethoprim, and penicillin.  Other patients may
be treated with a combination of antibiotics including tetracycline,
streptomycin, and penicillin or trimethoprim and sulfamethoxazole.
Antibiotic therapy may be necessary for a few months to several years.  In
rare cases with severe symptoms associated with Whipple's Disease,
corticosteroid drugs (e.g., prednisone) may be added to the antibiotic
regimen.

Some people with severe intestinal malabsorption caused by Whipple's
Disease may require the intravenous administration of fluids and
electrolytes.  Other patients may require iron, folate supplements, vitamin
D, and calcium.  Since most patients with this disorder suffer from
malnutrition, the recommended diet is usually high in calories and protein.
The diet should be monitored regularly by a physician.

While the symptoms of Whipple's Disease may improve rapidly with long-
term antibiotic therapy, biopsy may reveal bacteria in the small intestine
for up to two years.  Whipple's Disease has been completely reversed by
antibiotic therapy.  The absence of bacilliform (rod shaped bacteria) in a
biopsy sample of the small bowel typically suggests remission and possible
cure.

Therapies:  Investigational

This disease entry is based upon medical information available through June
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 Resources section for the most current
information about this disorder.

Resources

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

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

     National Digestive Diseases Information Clearinghouse
     Box NDIC
     Bethesda, MD  20892
     [phone]

     Centers for Disease Control (CDC)
     1600 Clifton Road, NE
     Atlanta, GA  30333
     [phone]

References

CECIL TEXTBOOK OF MEDICINE, 19th Ed.:  James B. Wyngaarden, and Lloyd H.
Smith, Jr., Editors; W.B. Saunders Co., 1990.  Pp. 698, 1560.

THE MERCK MANUAL, 16th Ed.:  Robert Berkow Ed.; Merck Research
Laboratories, 1992.  Pp. 824-5, 829, 801.

GASTROINTESTINAL DISEASE, 4TH Ed.; Marvin H. Sleisenger, M.D. et al.;
W.B. Saunders Co., 1989.  Pp. 1302-06.

WHIPPLE'S DISEASE, FAMILIAL MEDITERRANEAN FEVER, AND ADULT-ONSET STILL'S
DISEASE.  A. McMenemy; Curr Opin Rheumatol (Aug 1991; 3(4)).  Pp. 597-600.

IDENTIFICATION OF THE UNCULTURED BACILLUS OF WHIPPLE'S DISEASE.  D.A.
Relman; N Engl J Med (Jul 30, 1992;327(5)).  Pp. 293-301.

SHORT-TERM ANTIBIOTIC TREATMENT IN WHIPPLE'S DISEASE.  J.C. Bai; J Clin
Gastroenterol (Jun 1991; 13(3)).  Pp. 303-7.