Whipple's Disease

Overview

What is Whipple’s disease?

Whipple’s disease is a rare bacterial infection. It affects how the small intestine processes fat and absorbs vitamins and nutrients. It can lead to malabsorption (lack of nutrients).

The disease can also affect the nervous system, joints and other organs like the heart and lungs. Exposure to Tropheryma whipplei bacteria found in soil and water causes the disease.

How common is Whipple’s disease?

Whipple’s disease is extremely rare. Approximately 10 out of 1 million Americans get Whipple’s disease every year.

Who might get Whipple’s disease?

Anyone can get Whipple’s disease. But the condition mostly affects white male adults over age 50.

Symptoms and Causes

What causes Whipple’s disease?

Tropheryma whipplei bacteria cause Whipple’s disease. This bacteria can be found everywhere in the environment, but infection is very rare. Experts still don’t know why some people get the infection while others don’t. Scientists have found the bacteria in the saliva and stool of people who don’t have Whipple’s disease. These people are more likely to have a protein called the human leukocyte antigen B27 (HLA-B27). If these people come into contact with Tropheryma whipplei bacteria, they might be more likely to get Whipple’s disease.

People with Whipple’s disease often have immune system problems. These issues make it harder for the body to fight infections.

Is Whipple disease contagious?

Whipple disease isn’t contagious like a cold or the flu. You can’t catch it from being near someone who is infected.

What are the symptoms of Whipple’s disease?

Whipple’s disease tends to affect the digestive system, specifically the small intestine. But it can also affect bones, joints, other organs and the nervous system.

The most common symptoms of Whipple’s disease include:

Between 20% to 40% of people with Tropheryma whipplei infection experience neurological problems. When Whipple’s disease affects the nervous system, you may have:

Other signs of Whipple’s disease include:

Diagnosis and Tests

How is Whipple’s disease diagnosed?

People with Whipple’s disease have the Tropheryma whipplei bacteria in their small intestines. The only way to test for the bacteria is through a biopsy.

Your healthcare provider uses a scope to remove a small piece of tissue from the small intestine. If you have a swollen lymph node, your provider may perform a needle biopsy through the skin. An expert in a lab examines the tissue under a microscope to check for bacteria.

Management and Treatment

What are the complications of Whipple’s disease?

Untreated Whipple’s disease can get worse and cause death. Malabsorption occurs when your body can’t process fat or other nutrients. As a result, your body lacks nutrients and vitamins.

Whipple’s disease can cause fluid to build up between thin membranes (pleura) that line the outside of the lungs. This condition, pleural effusion, causes chest pain and shortness of breath. Your healthcare provider may need to drain the fluid.

A heart murmur (abnormal blood flow in the heart) is another potential complication.

How is Whipple’s disease managed or treated?

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

  • Antibiotics: You may start with intravenous (IV) antibiotics. Most people take several different types and doses of antibiotics by mouth for up to one year. Some people may take antibiotics by mouth plus hydroxychloroquine.
  • Fluid replacement: You may receive IV solutions to prevent dehydration. Or you may drink electrolyte beverages to replenish lost fluids and salt.
  • Vitamins and nutrients: Your provider may recommend taking supplements to make up for vitamins and nutrients your intestines can’t absorb.

Prevention

How can I prevent Whipple’s disease?

Wearing gloves when working in outdoor soil and water sources is one way to prevent Whipple's disease. You may also consider wearing a mask for special situations (for example, sewage workers). You should wash your hands thoroughly after working in dirt, mud or dirty water.

Outlook / Prognosis

What is the prognosis (outlook) for people who have Whipple’s disease?

Most people get symptom relief within one month of starting treatment and do well long-term. But it can take as long as two years for the small intestine to recover fully. Relapses (a return of symptoms) are common. After one year of treatment, your healthcare provider may perform another biopsy to check for the bacteria.

Whipple’s disease that affects the nervous system can cause lifelong problems. The condition is sometimes fatal without treatment.

Living With

When should I call the doctor?

You should call your healthcare provider if you experience:

  • Balance or coordination problems.
  • Chest pain or shortness of breath.
  • Joint pain.
  • Loss of appetite or unintended weight loss.
  • Seizures.
  • Severe abdominal pain or diarrhea.
  • Vision, speech or swallowing problems.

What questions should I ask my doctor?

You may want to ask your healthcare provider:

  • What are treatment side effects?
  • How can I manage treatment side effects?
  • Should I increase fluids or electrolytes?
  • Should I take vitamin supplements?
  • Should I lookout for signs of complications?

A note from Cleveland Clinic

Whipple’s disease is extremely rare. Antibiotics treat this bacterial infection, but the recovery can be long. For some people, symptoms return while taking antibiotics or soon after treatment ends. Your healthcare provider will monitor your health and start new medicines if symptoms return. Rarely, Whipple’s disease affects the nervous system and causes brain damage. Left untreated, Whipple’s disease can be fatal.

Last reviewed by a Cleveland Clinic medical professional on 06/11/2021.

References

  • Dutly F, Altwegg M. . Clin Microbiol Rev. 2001;14(3):561-583. Accessed 6/23/2021.Whipple’s disease and Tropheryma whipplii (https://journals.asm.org/doi/full/10.1128/CMR.14.3.561-583.2001)
  • Elchert JA, Mansoor E, Abou-Saleh M, Cooper GS. . Dig Dis Sci. 2019;64(5):1305-11. Accessed 6/23/2021.The epidemiology of Whipple’s disease in the United States between 2012 and 2017: A population based national study (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6499665/)
  • Hujeol IA, Johnson DH, Lebwohl B, et al. Tropheryma whipplei Infection (Whipple Disease) in the USA. Dig Dis Sci. 2019 Jan;64(1):213-223.
  • Kloeck AT, Piet JR, Adriani KS. . Neurology. 2020;94(7):e758-e761. Accessed 6/23/2021.Pearls and oysters: A rare presentation of Whipple disease (https://n.neurology.org/content/94/7/e758)
  • Merck Manual. . Accessed 6/23/2021.Whipple Disease (https://www.merckmanuals.com/home/digestive-disorders/malabsorption/whipple-disease)
  • National Institute of Health Genetic and Rare Diseases Information Center. . Accessed 6/23/2021.Whipple Disease (https://rarediseases.info.nih.gov/diseases/7889/whipple-disease)

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