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Whipple disease

Definition

Whipple disease is a rare condition that mainly affects the small intestine. This prevents the small intestines from allowing nutrients to pass into the rest of the body. This is called malabsorption.

Alternative Names

Intestinal lipodystrophy

Causes

Whipple disease is caused by infection with a form of bacteria called Tropheryma whipplei. The disorder mainly affects white men of middle-age.

Whipple disease is very 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:

Exams and Tests

The health care provider will perform a physical exam. This may show:

Tests to diagnose Whipple disease may include:

  • Complete blood count (CBC)
  • Polymerase chain reaction (PCR) test to check for the bacteria that cause the disease
  • Small bowel biopsy
  • Upper GI endoscopy (viewing the intestines with a flexible, lighted tube in a process called enteroscopy)

This disease may also change the results of the following tests:

Treatment

People with Whipple disease need to take long-term antibiotics to cure any infections of 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)
  • Nutritional deficiencies
  • Symptoms return (which may be because of drug resistance)
  • Weight loss

When to Contact a Medical Professional

Call your provider if you have:

  • Joint pain that does not go away
  • Abdominal pain
  • Diarrhea

If you are being treated for Whipple disease, call your provider if:

  • Symptoms get worse or do not improve
  • Symptoms reappear
  • New symptoms develop

References

Maiwald M, von Herbay A, Relman DA. Whipple's disease. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease. 10th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 109.

Marth T, Schneider T. Whipple's disease. In: Bennett JE, Dolin R, Blaser MJ, eds. Mandell, Douglas, and Bennett's Principles and Practice of Infectious Diseases, Updated Edition. 8th ed. Philadelphia, PA: Elsevier Saunders; 2015:chap 212.

West SG. Systemic diseases in which arthritis is a feature. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 275.


Review Date: 4/2/2018
Reviewed By: Michael M. Phillips, MD, Clinical Professor of Medicine, The George Washington University School of Medicine, Washington, DC. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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