Overview
Whipple disease is a rare bacterial infection that most often affects your joints and digestive system. Whipple disease interferes with normal digestion by impairing the breakdown of foods, and hampering your body's ability to absorb nutrients, such as fats and carbohydrates.
Small intestine lining
The small intestine has a ridged lining covered with tiny fingerlike projections called villi.
Whipple disease can also infect other organs, including your brain, heart and eyes.
Without proper treatment, Whipple disease can be serious or fatal. However, a course of antibiotics can treat Whipple disease.
Symptoms
Common signs and symptoms
Digestive signs and symptoms are common in Whipple disease and may include:
- Diarrhea
- Stomach cramping and pain, which may worsen after meals
- Weight loss, associated with the malabsorption of nutrients
Other frequent signs and symptoms associated with Whipple disease include:
- Inflamed joints, particularly the ankles, knees and wrists
- Fatigue
- Weakness
- Anemia
Less common signs and symptoms
In some cases, signs and symptoms of Whipple disease may include:
- Fever
- Cough
- Enlarged lymph nodes
- Skin darkening in areas exposed to the sun and in scars
- Chest pain
Brain and nervous system (neurological) signs and symptoms may include:
- Difficulty walking
- Vision problems, including lack of control of eye movements
- Confusion
- Memory loss
Symptoms tend to develop slowly over many years in most people with this disease. In some people, symptoms such as joint pain and weight loss develop years before the digestive symptoms that lead to diagnosis.
When to see a doctor
Whipple disease is potentially life-threatening yet usually treatable. Contact your doctor if you experience unusual signs or symptoms, such as unexplained weight loss or joint pain. Your doctor can perform tests to determine the cause of your symptoms.
Even after the infection is diagnosed and you're receiving treatment, let your doctor know if your symptoms don't improve. Sometimes antibiotic therapy isn't effective because the bacteria are resistant to the particular drug you're taking. The disease can recur, so it's important to watch for symptoms that reappear.
Causes
Whipple disease is caused by a type of bacterium called Tropheryma whipplei. The bacteria affect the mucosal lining of your small intestine first, forming small sores (lesions) within the wall of the intestine. The bacteria also damage the fine, hairlike projections (villi) that line the small intestine.
Not much is known about the bacteria. Although they seem readily present in the environment, scientists don't know where they come from or how they're spread to humans. Not everyone who carries the bacteria develops the disease. Some researchers believe that people with the disease may have a genetic defect in their immune system response that makes them more likely to become sick when exposed to the bacteria.
Whipple disease is extremely uncommon, affecting fewer than 1 in 1 million people.
Risk factors
Because so little is known about the bacteria that cause Whipple disease, risk factors for the disease haven't been clearly identified. Based on available reports, it appears more likely to affect:
- Men ages 40 to 60
- White people in North America and Europe
- Farmers and other people who work outdoors and have frequent contact with sewage and wastewater
Complications
The lining of your small intestine has fine, hairlike projections (villi) that help your body absorb nutrients. Whipple disease damages the villi, impairing nutrient absorption. Nutritional deficiencies are common in people with Whipple disease and can lead to fatigue, weakness, weight loss and joint pain.
Whipple disease is a progressive and potentially fatal disease. Although the infection is rare, associated deaths continue to be reported. This is due in large part to late diagnoses and delayed treatment. Death often is caused by the spread of the infection to the central nervous system, which can cause irreversible damage.
