Home Health Topics What Is Whipple Disease? Symptoms, Causes, Diagnosis, Treatment, and Prevention

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What Is Whipple Disease? Symptoms, Causes, Diagnosis, Treatment, and Prevention

June 23, 2019

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Whipple disease is a rare bacterial infection that interferes with the body’s ability to process nutrients. The disease usually occurs in the gastrointestinal system, particularly the small intestine, but it can affect these other parts of the body:

Eyes
Heart
Lungs
Joints
Central nervous system (the brain and spinal cord)
Nerves throughout the body

With antibiotic treatment, Whipple disease can generally be cured. But if left untreated, it can lead to serious complications, such as long-lasting nutritional deficiencies as well as heart and brain damage, and can be fatal.

Causes and Risk Factors of Whipple Disease

Whipple disease is caused by the bacteria Tropheryma whipplei. Scientists don’t know exactly how people get infected with T. whipplei, but this bacteria seems to be common in soil and water. (Not surprisingly, many reports of Whipple disease occur in individuals who work in the agricultural or construction sector and whose jobs require them to have frequent contact with soil.

According to a review published in April 2017 in the journal Clinical Microbiology Reviews, while Whipple disease is rare, the T. whipplei bacteria is more common than previously believed. The review authors cite research that shows up to 48 percent of the general population in Europe carries antibodies against T. whipplei, and the number is even higher in Senegal — 72 percent. (The review states that a high presence of the bacteria in African and Asian populations is particularly noteworthy given the fact that Whipple disease is less common in those groups compared with Caucasian populations.)

The review also cites recent data that suggests T. whipplei has been detected in Europe in 1.5 to 4 percent of fecal samples from asymptomatic people in the general population compared with as many as 12 to 25 percent of samples taken from sewage workers, people infected with HIV, and those experiencing homelessness, indicating that these groups have a higher risk of developing Whipple disease when exposed to the bacteria.

In other words, it appears that not everyone who carries T. whipplei develops Whipple disease. Some researchers believe that certain people may have a genetic susceptibility that makes them more likely to become sick when they’re exposed to the bacteria.

How Is Whipple Disease Diagnosed?

To diagnose Whipple disease, your doctor will begin by getting your medical history and conducting a physical examination, which may involve checking for enlarged lymph glands, a heart murmur, or swelling in your body tissues. Your doctor will use your symptoms to guide which diagnostic tests to order.

Initial assessments may involve:

Blood tests to check for anemia, abnormal electrolyte levels, liver function, and nutrient malabsorption
Blood tests for inflammatory joint disease if you have joint aches or swelling
Stool studies if you have diarrhea
Gastrointestinal endoscopy (a procedure in which the intestines are viewed using a lighted, flexible tube)

A definitive diagnosis of Whipple disease requires a biopsy of an affected organ — in which a tissue sample is viewed under a microscope — as well as a PCR (polymerase chain reaction) test on the tissue. Depending on the results of your exam and tests, your doctor may refer you to a gastroenterologist, a doctor who specializes in digestive diseases.

Prognosis of Whipple Disease

With Whipple disease, the earlier the diagnosis and treatment, the better the prognosis. While Whipple disease can be treated, and most people with the condition will likely have a good outcome, relapse is common. This is why it’s important for someone who has been treated for Whipple disease to be closely monitored and to undergo endoscopy with biopsy and other diagnostic tests a year after their first diagnosis and treatment.

People who experience neurological symptoms are at a greater risk of experiencing negative health outcomes such as serious neurological problems and even death.This is why early diagnosis is crucial for Whipple disease. If untreated, Whipple disease will progressively get worse and is most often fatal.

Treatment and Medication Options for Whipple Disease

Treatment for Whipple disease typically involves taking antibiotics for a prolonged period of time — at least one to two years — to eradicate the bacteria that's causing the illness. And depending on how much of a toll the intestinal malabsorption has taken on an individual’s body, doctors may recommend IV fluids and electrolytes (minerals in the body, such as sodium and potassium, that the heart and brain need to function properly) as well as supplements such as iron, folate, calcium, and vitamin D.

Doctors may also recommend a diet that’s high in calories and protein to make up for the malnutrition commonly experienced by people with Whipple disease.

Medication Options

If you have Whipple disease, your doctor will first prescribe a two-week round of these types of IV antibiotics:

ceftriaxone
meropenem (Merrem)
penicillin G
streptomycin

After this initial treatment, you'll have to take oral antibiotics for one to two years to make sure your body is clear of T. whipplei. These medications may include the following:

trimethoprim with sulfamethoxazole (Septra, Bactrim), a combination antibiotic
doxycycline (Vibramycin)

Some individuals experience a relapse with worsening neurological symptoms, which may be treated with antibiotics and injections of interferon gamma, a protein that activates the immune system.

Prevention of Whipple Disease

At this time, doctors do not have a way of preventing Whipple disease.

Research and Statistics: Who Has Whipple Disease?

The use of PCR testing as a tool to diagnose Whipple disease has made it possible for scientists to identify cases of Whipple disease that may have been previously missed.

According to recent studies cited in an editorial published in November 2018 in Digestive Diseases and Sciences, the prevalence of Whipple disease has been estimated to be 3 in 1 million people in Western populations. But cases may be underreported, suggest the authors, since criteria for diagnosis varies.

Some noteworthy facts and numbers about Whipple disease:

People of any age can get Whipple disease, but symptoms typically begin between age 30 and 60.
The median age of diagnosis is 56.
Whipple disease is more common in men — 4 times as many men get this illness compared with women.

Related Conditions of Whipple Disease

A number of conditions have symptoms and signs that are similar to those of Whipple disease:

Crohn’s Disease This inflammatory bowel disease causes chronic inflammation of the gastrointestinal tract. Symptoms can include diarrhea, blood in the stool, fever, weight loss, and abdominal pain.
Ulcerative Colitis Another type of inflammatory bowel disease, ulcerative colitis can lead to diarrhea, bloody stools, and abdominal pain. Other symptoms include cramping and an urgent need to empty the bowels.
Rheumatoid Arthritis This autoimmune disorder causes joint stiffness, weakness, fatigue, and painful swelling in the joints.

Resources We Love

National Organization for Rare Disorders (NORD)

This site is a go-to source for information on disorders that are not common but can profoundly affect individuals who are affected by them. From facts about symptoms and causes to who might be affected and which latest treatments might be right and why, NORD, which also provides links to other resources, is a good place to start to get more information about Whipple disease.

National Institute of Neurological Disorders and Stroke (NINDS)

In addition to helpful information about Whipple disease, such as symptoms, treatment, and prognosis, the NINDS website also links to clinical trials related to this rare disease.

Additional reporting by Joseph Bennington-Castro.

Editorial Sources and Fact-Checking

Whipple’s Disease Information Page. National Institute of Neurological Disorders and Stroke. March 27, 2019.
Whipple Disease. National Organization for Rare Disorders. 2009.
Whipple Disease. MedlinePlus. January 5, 2021.
Whipple Disease. Genetic and Rare Diseases Information Center.
Sellin J, Beales ILP. Whipple’s Disease: A Well-Done Outcome to a Rare Disease. Digestive Diseases and Sciences. November 2018.
Antunes C, Singhal M. Whipple Disease. StatPearls. July 2020.
Dolmans RAV, Boel CHE, Lacle MM, Kusters JG. Clinical Manifestations, Treatment, and Diagnosis of Tropheryma whipplei Infections. Clinical Microbiology Reviews. April 2017.
Whipple’s Disease. Mayo Clinic. December 9, 2020.
Glaser C, Rieg S, Wiech T, et al. Whipple’s Disease Mimicking Rheumatoid Arthritis Can Cause Misdiagnosis and Treatment Failure. Orphanet Journal of Rare Diseases. May 2017.