Whipple disease
Intestinal lipodystrophy; Whipple's diseaseWhipple disease is a rare condition that mainly affects the small intestine. This prevents the small intestine from allowing nutrients to pass into the rest of the body. This is called malabsorption.
Malabsorption
Malabsorption involves problems with the body's ability to take in (absorb) nutrients from food.
Read Article Now Book Mark ArticleCauses
Whipple disease is caused by infection with a bacterium called Tropheryma whipplei. The disorder mainly affects white men of middle-age.
Whipple disease is rare. Risk factors are not known.
Symptoms
Symptoms most often start slowly. Joint pain is the most common early symptom. Symptoms of gastrointestinal (GI) infection often occur several years later. Other symptoms may include:
- Abdominal pain
Abdominal pain
Abdominal pain is pain that you feel anywhere between your chest and groin. This is often referred to as the stomach region or belly.
Read Article Now Book Mark Article - Diarrhea
- Fever
- Darkening of skin in light-exposed areas of the body
- Joint pain in the ankles, knees, elbows, fingers, or other areas
- Memory loss
- Mental changes
Mental changes
Confusion is the inability to think as clearly or quickly as you normally do. You may feel disoriented and have difficulty paying attention, remembe...
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Exams and Tests
The health care provider will perform a physical exam. This may show:
- Enlarged lymph glands
Enlarged lymph glands
Lymph nodes are present throughout your body. They are an important part of your immune system. Lymph nodes help your body recognize and fight germ...
Read Article Now Book Mark Article - Heart murmur
Heart murmur
A heart murmur is a blowing, whooshing, or rasping sound heard during a heartbeat. The sound is caused by turbulent (rough) blood flow through the h...
Read Article Now Book Mark Article - Swelling in body tissues (edema)
Edema
Swelling is the enlargement of organs, skin, or other body parts. It is caused by a buildup of fluid in the tissues. The extra fluid can lead to a ...
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Tests to diagnose Whipple disease may include:
- Complete blood count (CBC)
CBC
A complete blood count (CBC) test measures the following:The number of white blood cells (WBC count)The number of red blood cells (RBC count)The numb...
Read Article Now Book Mark Article - Polymerase chain reaction (PCR) test to check for the bacteria that cause the disease
- Small bowel biopsy
Biopsy
A biopsy is the removal of a small piece of tissue for laboratory examination.
Read Article Now Book Mark Article - Upper GI endoscopy
Endoscopy
Endoscopy is a way of looking inside the body using a flexible tube that has a small camera and light on the end of it. This instrument is called an...
Read Article Now Book Mark Article - Small bowel enteroscopy
Enteroscopy
Enteroscopy is a procedure used to examine the small intestine (small bowel).
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This disease may also change the results of the following tests:
- Albumin levels in the blood
Albumin
Albumin is a protein made by the liver. A serum albumin test measures the amount of this protein in the clear liquid portion of the blood. Albumin c...
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Unabsorbed fat in the stools (fecal fat)
Fecal fat
The fecal fat test measures the amount of fat in the stool. This can help gauge the percentage of dietary fat that the body does not absorb....
Read Article Now Book Mark Article - Intestinal absorption of a type of sugar (d-xylose absorption)
D-xylose absorption
D-xylose absorption is a laboratory test to check how well the intestines absorb a simple sugar (D-xylose). The test helps detect if nutrients are b...
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Treatment
People with Whipple disease need to take long-term antibiotics to cure the infection that may include sites in the brain and central nervous system. An antibiotic called ceftriaxone is given through a vein (IV). It is followed by another antibiotic (such as trimethoprim-sulfamethoxazole) taken by mouth for up to 1 year.
If symptoms come back during antibiotic use, the medicines may be changed.
Your provider should closely follow your progress. Symptoms of the disease can return after you finish the treatments. People who remain malnourished will also need to take dietary supplements.
Outlook (Prognosis)
If not treated, the condition is most often fatal. Treatment relieves symptoms and can cure the disease.
Possible Complications
Complications may include:
- Brain damage
- Heart valve damage (from endocarditis)
Endocarditis
Endocarditis is inflammation of the inside lining of the heart chambers and heart valves (endocardium). It is caused by a bacterial or, rarely, a fu...
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- Symptoms return (which may be because of drug resistance)
- Weight loss
Weight loss
Unexplained weight loss is a decrease in body weight, when you did not try to lose the weight on your own. Many people gain and lose weight. Uninten...
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When to Contact a Medical Professional
Contact your provider if you have:
- Joint pain that does not go away
- Abdominal pain
- Diarrhea
If you are being treated for Whipple disease, contact your provider if:
- Symptoms get worse or do not improve
- Symptoms reappear
- New symptoms develop
References
Maiwald M, von Herbay A, Relman DA. Whipple disease. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease. 11th ed. Philadelphia, PA: Elsevier; 2021:chap 109.
Marth T, Schneider T. Whipple disease. In: Bennett JE, Dolin R, Blaser MJ, eds. Mandell, Douglas, and Bennett’s Principles and Practice of Infectious Diseases. 9th ed. Philadelphia, PA: Elsevier; 2020:chap 210.
West SG. Systemic diseases in which arthritis is a feature. In: Goldman L, Cooney KA, eds. Goldman-Cecil Medicine. 27th ed. Philadelphia, PA: Elsevier; 2024:chap 254.
Review Date: 5/14/2024
Reviewed By: Jenifer K. Lehrer, MD, Department of Gastroenterology, Aria - Jefferson Health Torresdale, Jefferson Digestive Diseases Network, Philadelphia, PA. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.