Definition of Whipple's disease:

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

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. 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
• Memory loss
• Mental changes
• Weight loss

Signs and tests:

Possible signs:

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

Tests to diagnose Whipple's disease may include:

• Complete blood count (CBC)
• Polymerase chain reaction (PCR) testing of the affected tissue for Tropheryma whippelii
• 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.

Expectations (prognosis):

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

Complications:

• Brain damage
• Heart valve damage (from endocarditis)
• 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 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