Whipple’s disease is caused by Tropheryma whipplei (T. whipplei) bacteria, which prevents the body from absorbing fats and other nutrients. Though rare, it can be serious and affect multiple body systems.

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First discovered in 1907, Whipple’s disease damages the function of the villi in the small intestine, which are responsible for moving nutrients from ingested food throughout the body, providing energy for growth, tissue repair, and more. This results in malabsorption.

Once the disease progresses beyond the digestive system, it can affect multiple organs and body systems, such as:

  • heart
  • lungs
  • brain
  • joints
  • eyes

Whipple’s disease can be life threatening, but the condition is relatively rare, affecting only 1-3 people per million around the world. It is slightly more common in the United States, affecting 9.8 out of every 1 million people based on data collected between 2012 and 2017.

The typical age of onset is 55 years old, and males are much more likely to be diagnosed than females at a rate of 4 to 1.

Are sex and gender the same thing?

People often use the terms sex and gender interchangeably, but they have different meanings:

  • “Sex” refers to the physical characteristics that differentiate male, female, and intersex bodies.
  • “Gender” refers to a person’s identity and how they feel inside. Examples include man, woman, nonbinary, agender, bigender, genderfluid, pangender, and trans. A person’s gender identity may be different from the sex they were assigned at birth.

Read more about sex and gender in this Healthline article.

Whipple’s disease can affect many different body parts and is associated with various symptoms.

The most common signs and symptoms of Whipple’s disease include gastrointestinal symptoms such as weight loss, diarrhea, and abdominal pain. About 80% of people will also experience intestinal bleeding.

You may also experience fever, swelling in the lower hands and legs, and chronic joint pain and inflammation.

Other less common symptoms may include:

Infection with the T. whipplei bacteria is the one and only known cause of Whipple’s. The bacteria cause internal sores to develop and body tissues to thicken.

The villi are finger-like tissues that absorb nutrients in the small intestine. When the villi begin to thicken, their natural shape begins to change. This damages the villi and prevents them from effectively absorbing nutrients. This leads to many of the symptoms of Whipple’s disease.

That said, many people who contract the bacterium may not have any symptoms. In people predisposed to Whipple’s disease, the immune response to the bacteria is muted, increasing their risk of developing the illness.

Whipple’s disease is more common in people who are white, non-Hispanic, or those over 65 years old.

T. whipplei bacterium is known to live in soil, so the disease can be more common among farmers.

However, there is also evidence of person-to-person transmission, as well as through waste water. The rate for Whipple’s disease also tends to be higher in places that lack fresh water and proper sanitation.

Currently, there’s no known way to prevent Whipple’s disease. Even so, very few people who contract the bacterium will develop the disease.

A diagnosis of Whipple’s disease is complicated, especially because symptoms are similar to other more common conditions, which range from celiac disease to neurological disorders.

A doctor will try to rule out these other conditions before diagnosing you with Whipple’s disease. They may refer you to the following procedures or tests.

Endoscopy

The first sign your doctor will look for to determine if you have Whipple’s disease is lesions.

An endoscopy involves the insertion of a small flexible tube down your throat to the small intestine. The tube has a mini camera attached.

Your doctor will observe the condition of your intestinal walls. Thick walls with creamy, ragged covers are a potential sign of Whipple’s.

Biopsy

During an endoscopy, your doctor may remove tissue from your intestinal walls to test for the presence of the T. whipplei bacteria. This procedure is called a biopsy and can confirm an infection.

Polymerase chain reaction (PCR)

Polymerase chain reaction (PCR) is a highly sensitive test that amplifies the DNA of the T. whipplei from your tissue samples. If the bacteria have been in your tissue, there will be DNA evidence.

This test can be used to confirm the existence of the T. whipplei bacteria in your tissue.

Blood tests

Your doctor might order a complete blood count. This will help them determine if you have a low count of red blood cells and low amounts of albumin, which are both signs of anemia.

Anemia is an indication that you might have Whipple’s disease.

An aggressive course of antibiotics is usually the first step in treatment, including 2–4 weeks of antibiotics through intravenous (IV) administration. In addition, you’ll likely be on daily antibiotics for 1–2 years.

It’s not entirely clear which type of antibiotic is best for treating Whipple’s disease. Tetracycline had been a primary choice for a long time, but doctors found that many people would get the disease again and not respond well to secondary treatment. Penicillin or trimethoprim/sulfamethoxazole (TMP-SMX) achieved better results.

Tell your doctor if you have an allergy to a particular antibiotic, as they have many other options they can try, including:

Other treatment options include:

Whipple’s disease can lead to death if it’s not treated properly. However, after treatment begins, many of the symptoms will go away within a month.

Relapses are common. After you finish your course of antibiotics, you have a 9%–15% chance of the bacterium staying in your body, especially if you have nervous system symptoms.

Still, the best thing you can do is continue taking your antibiotics and finish your course. Afterward, you may want to be monitored regularly by your doctor. If you experience a relapse, you’re more likely to experience new symptoms, especially neurological ones.