Whipple disease

Definition

Whipple disease is a rare condition that mainly affects the small intestine. This prevents the small intestine from allowing nutrients to pass into the rest of the body. This is called malabsorption.

Alternative Names

Intestinal lipodystrophy

Causes

Whipple disease is caused by infection with bacterium called Tropheryma whipplei. The disorder mainly affects white men of middle-age.

Whipple disease is rare. Risk factors are not known.

Symptoms

Symptoms most often start slowly. Joint pain is the most common early symptom. Symptoms of gastrointestinal (GI) infection often occur several years later. Other symptoms may include:

• Abdominal pain
• Diarrhea
• Fever
• Darkening of skin in light-exposed areas of body
  
• Joint pain in the ankles, knees, elbows, fingers, or other areas
• Memory loss
• Mental changes
• Weight loss

Exams and Tests

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)

Treatment

People with Whipple disease need to take long-term antibiotics to cure the infection that may include sites in 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.

Outlook (Prognosis)

If not treated, the condition is most often fatal. Treatment relieves symptoms and can cure the disease.

Possible Complications

Complications may include:

• 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

Contact your provider if you have:

• Joint pain that does not go away
• Abdominal pain
• Diarrhea

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

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

References

Maiwald M, von Herbay A, Relman DA. Whipple disease. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease. 11th ed. Philadelphia, PA: Elsevier; 2021:chap 109.

Marth T, Schneider T. Whipple disease. In: Bennett JE, Dolin R, Blaser MJ, eds. Mandell, Douglas, and Bennett’s Principles and Practice of Infectious Diseases. 9th ed. Philadelphia, PA: Elsevier; 2020:chap 210.

West SG. Systemic diseases in which arthritis is a feature. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 259.