Whipple’s disease is a rare bacterial infection primarily affecting the small intestine. It can also affect the heart, lungs, brain, joints, and eyes.
Whipple’s disease is caused by bacteria called Tropheryma whipplei (T. whipplei).
Anyone can get Whipple’s disease, but it is more common in middle-aged Caucasian men.
Scientists are unsure how T. whipplei infects people. One theory is that some people are more vulnerable to Whipple’s disease due to genetic factors that influence the body’s immune system. This theory is supported by the existence of a relatively high number of people who have the bacteria in their bodies but don’t get sick. Also, the bacteria are more common in the environment (showing up in soil and sewage wastewater) than would be predicted based on the rareness of the disease. And while multiple cases of Whipple’s disease have occurred within the same family, no documentation exists of a person-to-person transmission.
T. whipplei infection can cause:
- Internal sores
- The thickening of tissues
- Villi (tiny finger-like projections that line the small intestine) take on an abnormal, clublike appearance. The damaged intestinal lining fails to properly absorb nutrients, causing diarrhea and malnutrition
Classic signs and symptoms of Whipple’s disease include:
- Periodic joint pain, with or without inflammation, that may persist for years before the appearance of other symptoms
- Chronic diarrhea, with or without blood
- Weight loss
- Abdominal pain and bloating
- Fever
- Fatigue
- Anemia — a condition in which the blood has a lower-than-normal number of red blood cells
Less common signs and symptoms of Whipple’s disease include:
- Darkening of the skin
- Enlarged lymph nodes
- Chronic cough
- Chest pain
- Pericarditis — inflammation of the membrane surrounding the heart
- Heart failure
Neurologic symptoms occur in some people diagnosed with Whipple’s disease and can mimic symptoms of almost any other neurologic condition. Neurologic symptoms of Whipple’s disease include:
- Vision problems
- Dementia
- Facial numbness
- Headache
- Muscle weakness or twitching
- Difficulty walking
- Memory problems
Symptoms of neurologic, lung, or heart disease occasionally appear without gastrointestinal symptoms. Left untreated, Whipple’s disease is fatal.
Whipple’s disease is treated with long-term antibiotics that kill T. whipplei bacteria. Standard therapy for Whipple’s disease involves initial treatment with intravenous (IV) antibiotics for 2 weeks, followed by daily oral antibiotic treatment for 1 to 2 years.
An alternative treatment for Whipple’s disease is a combination of doxycycline (Vibramycin) plus the antimalarial drug hydroxychloroquine (Plaquenil) taken for 12 to 18 months. Supporters of this approach recommend that people with neurologic Whipple’s disease also take long-term antibiotics that can enter the cerebrospinal fluid and brain, such as sulfamethoxazole.
After treatment, the likely outcome for most people with Whipple’s disease is good. Most symptoms disappear in about 1 month. Relapse is common, however, highlighting the need to closely watch for a return of symptoms.
People with neurologic Whipple’s disease who relapse tend to have much poorer health outcomes, including serious neurologic symptoms and even death; therefore, some scientists argue that all cases of Whipple’s disease should be considered neurologic. Relapsing neurologic Whipple’s disease is sometimes treated with a combination of antibiotics and weekly injections of interferon gamma (IFNγ)—a substance made by the body that activates the immune system.