What is Whipple’s disease?
Whipple’s disease is a rare bacterial infection primarily affecting the small intestine. It can also affect the heart, lungs, brain, joints, and eyes. Left untreated, Whipple’s disease is fatal.

What causes Whipple’s disease?
Bacteria called Tropheryma whipplei (T. whipplei) cause Whipple’s disease. T. whipplei infection can cause internal sores, also called lesions, and the thickening of tissues. Villi, which are tiny fingerlike projections that line the small intestine, take on an abnormal, club like appearance. The damaged intestinal lining fails to properly absorb nutrients, causing diarrhea and malnutrition.

Scientists are unsure how T. whipplei infects people. One theory is that some people are more vulnerable to Whipple’s disease—probably due to genetic factors that influence the body’s immune system. This theory is supported by the existence of a relatively high number of asymptomatic carriers—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.

Who gets Whipple’s disease?
Anyone can get Whipple’s disease, but it is more common in middle-aged Caucasian men.

What are the signs and symptoms of Whipple’s disease?
Signs and symptoms of Whipple’s disease vary widely.

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

Neuralgic symptoms occur in some people diagnosed with Whipple’s disease and can mimic symptoms of almost any other neuralgic condition.

Neurological symptoms of Whipple’s disease include
vision problems
dementia
facial numbness
headache
muscle weakness or twitching
difficulty walking
memory problems

Symptoms of neuralgic, lung, or heart disease occasionally appear without gastrointestinal symptoms.

How is Whipple’s disease treated?
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. IV antibiotics are delivered through a needle inserted into a vein. IV antibiotics used to treat Whipple’s disease include ceftriaxone (Rocephin) and penicillin G (Pfizer pen) plus streptomycin. Trimethoprim/sulfamethoxazole (Septra, Bactrim), a combination oral antibiotic that can enter the cerebrospinal fluid and brain, is commonly used to treat Whipple’s disease.

An alternative treatment for Whipple’s disease is a combination of doxycycline (Vibramycin) plus the ant malarial 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.
 

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