Whipple's Disease

What is Whipple’s Disease?

Whipple’s disease is a rare bacterial infection in your small intestine. It affects your body’s ability to digest food and absorb nutrients (malabsorption). You may develop Whipple’s disease after exposure to Tropheryma whipplei, a common bacterium that lives in dirt. Fortunately, a combination of antibiotics kills the bacteria in your body, eases your symptoms and cures the condition.

How common is Whipple’s disease?

While the bacterium that causes the disease is common, Whipple’s disease is very rare. Experts analyzing Whipple’s disease cases estimate it affects 9 in 1 million people in the U.S. Not everyone who encounters T.whipplei develops the disease.

What are the symptoms of Whipple’s disease?

Whipple’s disease symptoms develop in phases (stages), starting with joint pain that’s severe and may come and go. The pain typically affects your large joints — your ankles, elbows, hips, knees and shoulders. Healthcare providers may call this the prodromal phase. This phase may last seven or eight years before you develop more symptoms, including:

• Abdominal pain.
• Diarrhea.
• Edema (swelling).
• Fatigue.
• Fever.
• Patches of skin that are darker than your usual skin color.
• Swollen lymph nodes.
• Unexplained weight loss.

Between 20% and 40% of people with Whipple’s disease develop central nervous system issues that cause symptoms like:

• Balance problems.
• Confusion.
• Difficulty swallowing.
• Memory loss.
• Personality changes.
• Seizures.
• Speech impediments.

What causes Whipple’s disease?

Experts know that a type of bacteria often found in dirt causes Whipple’s disease. Anyone who encounters the bacteria could be at risk of infection. But Whipple’s disease is rare, and it’s not clear why more people don’t develop the disease after exposure.

Research suggests the following factors may increase your risk of developing the disease:

• Age: Whipple’s disease appears to affect people ages 65-84.
• Exposure to soil or livestock: Research suggests people whose work exposes them to soil or livestock have an increased risk of developing Whipple’s disease.
• Genetic mutation (change): There’s a connection between Whipple’s disease and the gene that makes the IFR4. This protein plays a role in developing B cells, T cells and other immune cells. Likewise, there’s an association between the disease and a mutation in the human leukocyte antigen B27 (HLA-B27) gene.
• Weak immune system: This can happen if you have a disease that affects your immune system or you take immunosuppressants.

What are the complications of Whipple’s disease?

Whipple’s disease affects your body’s ability to absorb fats, carbohydrates, vitamins and other nutrients from food that passes through your small intestine. It’s a progressive disease, meaning that it gets worse over time and causes new and more serious medical issues, including:

• Anemia (low red blood cells).
• Dementia.
• Heart murmur.
• Oculomasticatory myorhythmia, where your eyes and jaw constantly move side to side.
• Pleural effusion.
• Uveitis.

Whipple’s disease that affects your central nervous system can be fatal.

How do healthcare providers diagnose Whipple’s disease?

Healthcare providers do a physical examination. They’ll take a medical history and ask about current and past changes in your body. They may do the following tests:

• Complete blood count (CBC).
• Upper endoscopy.
• Polymerase chain reaction (PCR) test to check for T. whipplei bacteria.
• Small intestine biopsy.

How is Whipple’s disease treated?

It can take a long time to get rid of the bacteria that cause Whipple’s disease. Treatment includes:

• Antibiotics: Treatment typically involves receiving antibiotics by vein for two to four weeks. After that, you receive antibiotics that you take by mouth for at least the next 12 months.
• Fluid replacement: You may receive IV solutions to prevent dehydration. Or you may drink electrolyte beverages to replenish lost fluids and salt.
• Vitamins and nutrient supplements: Your provider may recommend that you take supplements to make up for vitamins and nutrients your intestines can’t absorb.

Treatment side effects

Antibiotics may cause the following side effects:

• Diarrhea.
• Dizziness.
• Nausea and vomiting.
• Rash.
• Yeast infections.

Can Whipple disease be prevented?

That’s hard to say. The bacteria that cause Whipple’s disease are everywhere in our environment, particularly in dirt. But not everyone who encounters them develops Whipple’s disease.

People with weak immune systems or who take immunosuppressants have an increased risk of developing the disease. If that’s your situation, it’s a good idea to wear gloves or to wash your hands thoroughly any time you work in dirt and mud.

What can I expect if I have Whipple’s disease?

If you’re like most people with Whipple’s disease, you’ll feel better soon after you start treatment. But it can take as long as two years for your small intestine to recover completely.

Treatment often cures Whipple’s disease, but it can come back after treatment. Your healthcare provider may recommend you have regular long-term follow-up tests. For example, they may recommend you have an upper endoscopy:

• Every six months during the first year of treatment.
• Once a year during the next three years.
• Once every three years for the rest of your life.

How do I take care of myself?

The most important thing is to take your antibiotics as prescribed. Most people will take antibiotics for at least a year after their diagnosis, and some people may need antibiotics for the rest of their lives.

If you have Whipple’s disease, you’ve been missing out on vital nutrients. Treatment will make you feel better, but you may want to ask a dietitian for recommendations on what you should eat and drink.

When should I see my healthcare provider?

Contact your provider if you still have Whipple’s disease symptoms after you start treatment or if your symptoms come back after treatment.

Why is this disease called Whipple’s disease?

The disease and the bacterium that causes it are named for George H. Whipple, a medical pathologist who first described the symptoms in 1907. More than 80 years later, researchers identified the bacterium that causes Whipple’s disease. They named it Tropheryma whipplei. The name honors Whipple’s work in discovering the disease and combines the Greek terms for nourishment (trophe) and barrier (eryma), or barrier to nourishment (malabsorption).

A note from Cleveland Clinic

There are bacteria all around us (and inside our bodies). In general, the bacteria we encounter daily don’t hurt us. The rare bacterial infection Whipple’s disease is an exception to that rule. Whipple’s disease happens after exposure to Tropheryma whipplei, a common bacteria that lives in dirt.

You may run into T. whipplei on the job or working in the yard. But not everyone who has exposure to it develops Whipple’s disease. And fortunately, prompt diagnosis and treatment often cure the condition.