Whipple disease (intestinal lipodystrophy) is the result of a rare bacterial infection that damages the lining of the small intestine and may involve other organs of the body.

• This disease is caused by a bacterial infection.
• Typical symptoms include diarrhea, inflamed and painful joints, fever, and skin darkening.
• 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. It is caused by an infection with the organism Tropheryma whippelii. The infection usually involves the small intestine but can affect other organs, such as the heart, lungs, brain, joints, and eyes.

Symptoms

Symptoms of Whipple disease include diarrhea, inflamed and painful joints, fever, and weight loss. Other common symptoms are abdominal pain, 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—see Pleural and Mediastinal Disorders: 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. If left untreated, the disease is progressive and fatal.

Diagnosis and Treatment

A doctor can make the diagnosis of Whipple disease by identifying bacteria in the small intestine or an enlarged lymph node. 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.

Whipple disease can be cured with antibiotics. Usually people are given ceftriaxone or penicillin initially by vein, followed by trimethoprim/sulfamethoxazole taken by mouth for at least 12 months. Symptoms subside rapidly. Despite an initial response to antibiotics, however, the disease can recur.

Last full review/revision January 2013 by Atenodoro R. Ruiz, Jr., MD