Whipple disease

What causes Whipple disease?

Whipple disease is caused by infection from bacteria called Tropheryma whipplei (or Tropheryma whippelii ). It is not known how people become infected with the bacteria, but there is no evidence of human to human transmission.

It seems that these bacteria are common in soil or water, and many reports of disease occur in patients who work in trades where they have frequent contact with soil such as agriculture and construction. The bacteria may also be part of the normal flora of the body. One study revealed its presence in saliva in 35% of a sample of 40 healthy patients. These findings, and the fact that Whipple disease is more common in people who have an antigen known as HLA-B27 suggest that there may be a genetic predisposition (susceptibility) in those people with the disease, resulting in an abnormal host response to a microorganism that occurs frequently in humans. In addition, most, if not all patients, have an immune defect which make it harder to fight infections.

Last updated on 05-01-20

What is the outlook for people with neurologic manifestations of Whipple disease?

After treatment, the likely outcome for most people with Whipple disease is good. Most symptoms disappear in about 1 month. Relapse is common, however, highlighting the need to closely watch for a return of symptoms. Endoscopy with small intestinal biopsy followed by PAS staining and electron microscopy or PCR testing should be repeated 1 year after the start of treatment.

People with neurologic Whipple disease who relapse tend to have much poorer health outcomes, including serious neurologic symptoms and even death; therefore, some researchers argue that all cases of Whipple disease should be considered neurologic.

Prompt diagnosis is imperative, as very effective therapies are easy to employ with typically rapid limitation of central nervous system (CNS) progression and even partial reversal of CNS symptoms. If left untreated, progression to death may come as quickly as 1 month after CNS involvement begins.

Last updated on 05-01-20

How might Whipple disease be treated?

The standard treatment for Whipple disease is a prolonged course of antibiotics to destroy the bacteria that cause the disease. A number of different types, doses, and schedules of antibiotics may be used in an effort to find the best treatment.

A medicine called ceftriaxone 2g, or penicillin G are commonly given intravenously as a first option. These antibiotics are typically followed by another antibiotic (such as trimethoprim- sulfamethoxazole) taken by mouth for up to 1 year. If symptoms come back during antibiotic use, the antibiotic treatment may be changed.

Depending on the seriousness of the disease, treatment may also include fluid and electrolyte replacement. Electrolytes are salts and other substances in body fluid that the heart and brain need to function properly. Extra iron, folate, vitamin D, calcium, and magnesium may also be given to help compensate for the vitamins and minerals the body cannot absorb on its own.

People who are being treated for Whipple disease should be closely monitored by a health care provider because signs of the disease can return after therapy has been finished. Full recovery of the small intestine may take up to 2 years, but the symptoms usually disappear in less time. Because relapse is common even after successful treatment, the health care team may continue to monitor the patient for many years.

In general, surgery is not needed, however, neurosurgical care is important for both obtaining diagnostic biopsy specimens in selected patients and placement of ventriculoperitoneal shunting in patients with hydrocephalus.

Last updated on 05-01-20


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The RareGuru disease database is regularly updated using data generously provided by GARD, the United States Genetic and Rare Disease Information Center.

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