Whipple's disease

Definition

Whipple's disease is a rare condition that prevents the intestine from properly absorbing nutrients. This is called malabsorption.

Alternative Names

Intestinal lipodystrophy

Causes, incidence, and risk factors

Whipple's disease is caused by infection from bacteria called Tropheryma whippelii. The disorder mainly affects middle-aged white men.

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

Symptoms

Symptoms usually start slowly, and may include:

• Abdominal pain
• Diarrhea
• Fever
• Gray to brown skin color
• Joint pain
• Memory loss
• Mental changes
• Weight loss

Signs and tests

Possible signs:

• Enlarged lymph glands
• Fatty stools
• Gastrointestinal bleeding
• Swelling in body tissues (edema)

Tests may include:

• Viewing the intestine with a flexible, lighted tube (enteroscopy), and possibly removing a small amount of intestinal lining (biopsy) to be examined under the microscope
• Complete blood count (CBC)

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

• Albumin
• Unabsorbed fat in the stools (fecal fat)
• Intestinal absorption of a type of sugar (d-xylose absorption)

Treatment

You will need to take long-term antibiotics to cure any infections of the brain and central nervous system. A medicine called ceftriaxone is given through a vein (IV). It is followed by an antibiotic (such as trimethoprim-sulfamethoxazole) taken by mouth for up to 1 year.

If symptoms come back during antibiotic use, the antibiotic treatment may be changed.

Your health care provider should closely follow you, because signs of the disease can return after you finish therapy. Those who have nutritional deficiencies from malabsorption will also need to take dietary supplements.

Expectations (prognosis)

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

Complications

• Nutritional deficiencies
• Symptoms return (which may be because of drug resistance)
• Weight loss

Calling your health care provider

Call your health care provider if you have persistent abdominal pain and diarrhea.

If you are being treated for Whipple's disease, call your health care provider if:

• Symptoms worsen or do not improve
• Symptoms reappear
• New symptoms develop

References

West SG. Systemic diseases in which arthritis is a feature. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007: chap 297.