Whipple's 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 different biopsies.
• If left untreated, the disease is progressive and fatal.
• Antibiotics can eliminate the infection, but the disease can recur.

Whipple's 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, lung, brain, joints, and eye.

Symptoms

Symptoms of Whipple's disease include diarrhea, inflamed and painful joints, fever, and skin darkening. Severe malabsorption results in weight loss along with fatigue and weakness caused by anemia. Other common symptoms are abdominal pain, 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 Disorders: Pleural Effusion). The lymph nodes may become enlarged. People with Whipple's 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's disease when an endoscopic biopsy (in which a tissue sample is obtained through a flexible tube and examined microscopically) of the small intestine or a biopsy of an enlarged lymph node shows the bacteria.

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

Last full review/revision December 2007 by Atenodoro R. Ruiz, Jr., MD