Overview

Whipple disease is a rare bacterial infection that most often affects your gastrointestinal system. Whipple disease interferes with normal digestion by impairing the breakdown of foods, such as fats and carbohydrates, and hampering your body's ability to absorb nutrients.

Whipple disease also can infect other organs, including your brain, heart, joints 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

Gastrointestinal signs and symptoms are common in Whipple disease and may include:

  • Diarrhea
  • Abdominal 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 (hyperpigmentation) in areas exposed to the sun and in scars
  • Chest pain
  • Enlarged spleen

Neurological signs and symptoms may include:

  • Difficulty walking
  • Visual impairment, including lack of control of eye movements
  • Confusion
  • Memory loss

Symptoms tend to develop slowly over a period of many years in most people with this disease. In some cases, some symptoms, such as joint pain and weight loss, develop years before the gastrointestinal 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 the re-emergence of symptoms.

Causes

The cause of Whipple disease is infection with the bacterium Tropheryma whipplei. This bacterium initially affects the mucosal lining of your small intestine, forming small lesions within the intestinal wall. The bacterium also damages the fine, hair-like projections (villi) that line the small intestine. With time, the infection can spread to other parts of your body.

Not much is known about the bacterium. Although it seems readily present in the environment, scientists don't really know where it comes from or how it's transmitted to humans. Not everyone who carries the bacterium 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 susceptible to becoming ill when exposed to the bacterium.

Whipple disease is extremely uncommon, affecting fewer than one in 1 million people.

Risk factors

Because so little is known about the bacterium that causes Whipple disease, risk factors for the disease haven't been clearly identified. Based on available reports, it appears more likely to affect:

  • Men
  • People ages 40 to 60
  • Whites 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, hair-like 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, 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.

Oct. 24, 2015
References
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