Whipple’s disease

Causes

Whipple's disease is caused by infection with a 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. Joint paint is the most common initial symptom. After that, often several years later, symptoms of gastrointestinal (GI) infection develop. Other symptoms may include:

• Abdominal pain
• Diarrhea
• Fever
• Gray to brown skin color
• Joint pain in the ankles, knees, elbows, fingers, or other areas
• Memory loss
• Mental changes
• Weight loss

Exams and Tests

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

• Enlarged lymph glands
• Heart murmur
• Swelling in body tissues (edema)

Tests to diagnose Whipple's disease may include:

• Complete blood count (CBC)
• Polymerase chain reaction (PCR) test to check for the bacteria that causes 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:

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

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 antibiotic treatment may be changed.

Your health care provider should closely follow your progress, 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.

Outlook (Prognosis)

Without treatment, the condition is usually 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 persistent joint pain, abdominal pain, or 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

Alternative Names

Intestinal lipodystrophy

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. Cecil Medicine. 24th ed. Philadelphia, PA: SaundersElsevier; 2011:chap 283.

Update Date: 5/1/2012

Updated by: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine. 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. Health Solutions, Ebix, Inc.