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Whipple Disease

(Whipple's Disease; Intestinal Lipodystrophy)

By

Atenodoro R. Ruiz, Jr.

, MD, The Medical City, Pasig City, Metro-Manila, Philippines

Last full review/revision Oct 2019| Content last modified Oct 2019
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Whipple disease is the result of a rare bacterial infection that damages the lining of the small intestine, resulting in malabsorption, and may involve other organs of the body.

  • This disease is caused by a bacterial infection.

  • Typical symptoms include diarrhea, inflamed and painful joints, weight loss, and abdominal pain.

  • The diagnosis is based on the results of biopsies of the small intestine.

  • If left untreated, the disease is progressive and fatal.

  • Antibiotics can eliminate the infection, but the disease can recur.

Whipple disease affects mainly white men aged 30 to 60.

Causes

Whipple disease is caused by an infection with the organism Tropheryma whipplei. The infection almost always affects the small intestine but can affect other organs, such as the heart, lungs, brain, joints, and eyes.

Symptoms

The four main symptoms of Whipple disease are

  • Inflamed and painful joints

  • Diarrhea

  • Abdominal pain

  • Weight loss

Other common symptoms are fever, loss of appetite, fatigue and weakness caused by anemia, cough, and pain when breathing caused by inflammation of the membrane layers covering the lungs (pleura). Fluid may collect in the space between the pleural layers (a condition called pleural effusion). The lymph nodes may become enlarged. In some people, the skin becomes darker. People with Whipple disease may develop heart murmurs. Confusion, memory loss, or uncontrolled eye movements indicate that the infection has spread to the brain.

Diagnosis

  • Biopsy

A doctor can make the diagnosis of Whipple disease by identifying bacteria in the small intestine.

To identify the bacteria, doctors remove tissue (biopsy) from the small intestine using an endoscope (a flexible viewing tube equipped with a light source and a camera through which a small clipper can be inserted) or remove tissue from an enlarged lymph node. The tissue is then examined under a microscope.

Treatment

  • Antibiotics

If left untreated, Whipple disease becomes progressively worse and is fatal.

Whipple disease can be cured with antibiotics. Usually people are given ceftriaxone or penicillin initially by vein for 2 to 4 weeks, followed by trimethoprim/sulfamethoxazole or a combination of doxycycline and hydroxychloroquine taken by mouth for at least 12 months. Symptoms subside rapidly. Despite an initial response to antibiotics, however, the disease can come back.

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