Whipple disease

Intestinal lipodystrophy

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

Whipple disease is very rare. Risk factors are unknown.

Symptoms most often start slowly. Joint pain is the most common early symptom. Symptoms of gastrointestinal (GI) infection often develop several years later. 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

The health care provider will perform a physical exam. This may show:

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

Tests to diagnose Whipple 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:

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

People with Whipple 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 medicines may be changed.

Your 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.

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

Complications may include:

• Brain damage
• Heart valve damage (from endocarditis)
• Nutritional deficiencies
• Symptoms return (which may be because of drug resistance)
• Weight loss

Call your provider if you have joint pain that does not go away, abdominal pain, or diarrhea.

If you are being treated for Whipple disease, call your provider if:

• Symptoms get worse or do not improve
• Symptoms reappear
• New symptoms develop