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Genetic and Rare Diseases Information Center (GARD)

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Whipple disease

Other Names for this Disease
  • Intestinal lipodystrophy
  • Intestinal lipophagic granulomatosis
  • Secondary Non-tropical Sprue
  • Tropheryma whippelii infection
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Your Question

Is there any reason for surgery in the management of Whipple disease?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is Whipple disease?

Whipple disease is a multi-system infectious bacterial disease that interferes with the body's ability to metabolize fats.[1] The disorder usually occurs in the gastrointestinal system, but may affect any part of the body including the heart, lungs, brain, joints, and eyes.[2] In the gastrointestinal system it interferes with the body's ability to absorb certain nutrients.[2][3] This leads to a condition known as malabsorption.[3] Whipple disease causes weight loss, incomplete breakdown of carbohydrates or fats, and malfunctions of the immune system.[2] It is caused by infection from bacteria called Tropheryma whippelii.[2][3] When recognized and treated, Whipple disease can usually be cured. Untreated, the disease may be fatal.[2]
Last updated: 8/1/2012

What causes Whipple disease?

Whipple disease is caused by infection from bacteria called Tropheryma whippelii.[2][3]
Last updated: 8/1/2012

What symptoms have been associated with Whipple disease?

The gastrointestinal symptoms of Whipple disease include diarrhea, intestinal bleeding, abdominal pain, loss of appetite, weight loss, fatigue, and weakness.[1][2] Arthritis and fever often occur several years before intestinal symptoms develop.[2] Patients may also experience neurological symptoms, including abnormalities of eye and facial muscle movements, confusion, seizures, ataxia, memory loss, and vision impairment.[1]

Last updated: 8/1/2012

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.[1][2][3] A number of different types, doses, and schedules of antibiotics may be utilized in an effort to find the best treatment.[2] A medicine called ceftriaxone is commonly given intravenously as a first round defense. It is typically followed by an 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.[3]

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.[2] 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.[2][3]

Individuals 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.[3] 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.[2]

Last updated: 8/1/2012

Is there any reason for surgery in the treatment of Whipple disease?

Surgery plays a very minor role in the management of individuals with Whipple disease. Neurosurgical care is relevant for both obtaining diagnostic biopsy specimens in selected patients and placement of ventriculoperitoneal shunting in patients with hydrocephalus. Both communicating and noncommunicating hydrocephalus has been reported in patients with Whipple disease affecting the central nervous system. The role of shunting in affected individuals should be thoroughly assessed.[4] 

Whipple disease bacillus can affect the periaqueductal gray matter, making noncommunicating hydrocephalus (secondary to aqueductal stenosis - an obstruction of the cerebral aqueduct) more likely in patients with advanced Whipple disease affecting the central nervous system. Patients with aqueductal stenosis represent a neurosurgical emergency and a ventriculoperitoneal shunt should be placed in an urgent manner.[4]

In progressive Whipple disease affecting the central nervous system with progressive, communicating hydrocephalus, it is possible to limit or reverse some of the cognitive and motor deficits by shunting. This is especially important as the reported reversal of other central nervous system symptoms and signs remains limited and requires several weeks to months despite otherwise efficient antibiotic treatment.[4]

Last updated: 8/1/2012