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, such as fats and carbohydrates, and hampering your body's ability to absorb nutrients.

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
  • 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 many years in most people with this disease. In some cases, 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 the re-emergence of symptoms.

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 lesions within the intestinal wall. 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 40-60
  • 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, 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. 26, 2018
References
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