Whipple Disease

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

Katherine Lee
By Medically Reviewed by 
Reviewed:
Medically Reviewed

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.

Signs and Symptoms of Whipple Disease

Symptoms of Whipple disease tend to vary from one person to another, but in general, symptoms can develop slowly, with joint pain commonly an early symptom and gastrointestinal problems occurring sometimes years later.

Typical signs and symptoms include:

  • Fatigue
  • Weakness
  • Anemia
  • Pain and stiffness in the joints, particularly in the ankles, knees, and fingers
  • Abdominal pain
  • Diarrhea
  • Fatty or bloody stools
  • Loss of appetite
  • Darkened skin
  • Enlarged lymph nodes
  • Chronic cough
  • Chest pain
  • Heart failure
People who have more advanced cases of Whipple disease may experience neurological difficulties. Up to 40 percent of people with Whipple disease may experience some of these symptoms:

Call your doctor if you notice the following:

  • Persistent joint pain
  • Abdominal pain
  • Symptoms that do not improve or reappear after treatment for Whipple disease

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.

While Whipple disease is rare, the T. whipplei bacteria is more common than previously believed. Research 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. (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.)

Data also 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.

Duration of Whipple Disease

Symptoms of Whipple disease can disappear about a month after antibiotic treatment.

However, the disease can cause complications related to malnutrition as well as heart and brain-related problems that can last for a prolonged period of time.

People who’ve had Whipple disease can also experience a relapse of symptoms, which can happen years after treatment.

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:

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:

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.

Complications of Whipple Disease

People with Whipple disease may experience complications due to the malnutrition caused by the damage to the small intestine. They may also experience common complications in other areas of their body, which can be worse due to delay in diagnosis or treatment:

  • Anemia (as many as 90 percent of people with Whipple disease experience anemia)

  • Weight loss
  • Nutritional deficiencies
  • Diarrhea
  • Joint pain
  • Heart problems (such as inflammation around the pericardium, called pericarditis, or inflammation of the heart’s inner lining, called endocarditis)
  • Neurologic problems such as slurred speech, confusion, seizures, delirium, cognitive impairment, or abnormal body movements
  • Return of Whipple disease symptoms during or after treatment (possibly due to antibiotic resistance)

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.

The prevalence of Whipple disease has been estimated to be 3 in 1 million people in Western populations. But cases may be underreported, 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

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

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.

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