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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:

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

Prevention:

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.

Review Date: 10/13/2008
Reviewed By: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and George F. Longstreth, MD, Department of Gastroenterology, Kaiser Permanente Medical Care Program, San Diego, California. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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