$Unique_ID{BRK04329} $Pretitle{} $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 } $Volume{} $Log{} 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 (203) 746-6518 National Digestive Diseases Information Clearinghouse Box NDIC Bethesda, MD 20892 (301) 468-2162 Centers for Disease Control (CDC) 1600 Clifton Road, NE Atlanta, GA 30333 (404) 639-3534 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.