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Whipple's disease

Whipple's disease is a rare condition that 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's disease is caused by infection with a form of bacteria called Tropheryma whippelii. The disorder mainly affects middle-aged white men.

Whipple's disease is very rare. Risk factors are unknown.

Symptoms

Symptoms most often start slowly. Joint paint is the most common early symptom. Symptoms of gastrointestinal (GI) infection often develop several years later. Other symptoms may include:

Exams and Tests

The doctor or nurse will perform a physical exam. This may show:

Tests to diagnose Whipple's 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's 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 medications may be changed.

Your health care 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)

Without treatment, the condition is most often fatal. Treatment relieves symptoms and can cure the disease.

Possible Complications


  • 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 health care provider if you have joint pain that does not go away, abdominal pain, or diarrhea.

If you are being treated for Whipple's disease, call your health care 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. 9th ed. Philadelphia, PA: Saunders Elsevier; 2010:chap 106.

West SG. Systemic diseases in which arthritis is a feature. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 24th ed. Philadelphia, PA: Saunders Elsevier; 2011:chap 283.

Updated: 5/15/2014

Jenifer K. Lehrer, MD, Department of Gastroenterology, Frankford-Torresdale Hospital, Aria Health System, Philadelphia, PA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.


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