When Discovered Early Whipple's Disease Can Often Be Treated With Antibiotics

Conor Loftus, M.D

DEAR MAYO CLINIC:

What is Whipple's Disease? My doctor said it might be related to my arthritis. How is it treated?

ANSWER:

Whipple's disease is a rare disorder caused by an infection. The small intestine is most commonly affected. But almost any organ system can be involved, including the joints. For someone who has Whipple's disease and arthritis, the two likely could be related. Prompt and aggressive antibiotic treatment typically cures the infection, relieving symptoms of the disease.

Whipple's disease results from infection with the microorganism Tropheryma whipplei. Although the microorganism apparently is common in the environment, researchers don't know exactly how Tropheryma whipplei is transmitted to humans. Most often, the disease is seen in middle-age men. For example, in one large series of 664 patients with the disease, 86 percent were male and the average age at diagnosis was 49. However, Whipple's disease is extremely uncommon. Estimates are that the disease affects less than one in 1 million people each year.

Usually, the first part of the body affected by Whipple's disease is the small intestine. The infection causes inflammation within the small intestine's lining and the surrounding lymph nodes. This inflammation leads to symptoms such as abdominal pain, cramping, diarrhea and weight loss. If not treated, Whipple's disease may lead to malnutrition because, due to chronic diarrhea, the small intestine is unable to absorb enough nutrients.

As the disease progresses, the infection can cause inflammation in almost any organ in the body, especially the nervous system, heart, joints, skin and eyes. Symptoms such as joint pain, back pain, double vision, difficulty with memory and thinking, altered consciousness and increased skin pigmentation can result. If the heart is affected, Whipple's disease can cause infection of the inner lining of the heart (endocarditis), infection of the heart muscle (myocarditis) and inflammation of the sac surrounding the heart (pericarditis).

Because Whipple's disease usually affects the small intestine first, the primary diagnostic test for this disorder is an endoscopic examination of the upper gastrointestinal tract with a tissue sample (biopsy) of the small intestine. During an upper endoscopy, a doctor who specializes in diseases of the digestive system (gastroenterologist) visually examines the upper digestive system with a tiny scope on the end of a long, flexible tube. For people who have symptoms of Whipple's disease involving other organs, a biopsy of those organs may also be necessary.

Treatment of Whipple's disease involves antibiotics to eradicate the microorganisms. In the past, oral antibiotics, such as tetracycline, were usually given first. With oral antibiotics, recurrence of the disease was not uncommon, often within the nervous system. As a result, the current recommendation is to treat people who have Whipple's disease with intravenous (IV) antibiotics for 10 to 14 days, followed by a course of oral antibiotics for one year. With this more aggressive treatment, the disease recurs less often.

Antibiotic treatment for Whipple's disease usually results in prompt and effective relief of symptoms, with resolution of diarrhea typically within days and joint pain and other symptoms within weeks.

Whipple's disease that recurs can be more difficult to treat successfully. Most people are now diagnosed and treated in the early stages of the disease, though, when treatment is more likely to be effective and recurrence following treatment is less common.

Conor Loftus, M.D., Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minn.

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