Definition

Whipple’s disease is a rare infectious disease. It is caused by a bacterium named Tropheryma whippleii . It can affect many parts of the body. The small intestine is the main organ affected. Other common areas include the joints, heart, nervous system, brain, lungs, eyes, and skin. Whipple’s disease occurs mainly in middle-aged men.

Causes

Whipple’s disease is caused by a specific type of bacteria. This bacterium causes abnormal changes on the wall of the small intestine. These changes disrupt the villi. Villi are tiny, finger-like structures that stick out from the wall of the intestine to help absorb nutrients. As a result, some nutrients are not absorbed properly and do not reach the rest of the body.

There is no evidence that Whipple’s disease is passed from person to person. However, there is some evidence that the bacteria may be present in people who are healthy. This suggests that the few people who develop the disease may have an abnormal response to the bacteria. Some people with Whipple’s disease may have a genetic defect that makes them more likely to develop the disease.

Cross Section of Intestine With Villi

Intestine Cross section
© 2009 Nucleus Medical Art, Inc.

Risk Factors

A risk factor is something that increases your chance of getting a disease or condition. Other than the possibility of a genetic defect, there are no known risk factors for Whipple’s disease.

Symptoms

Symptoms usually begin slowly and occur in stages. The first stage includes:

  • Pain in the joints (in 90% of cases)
  • Fever

The disease is usually not diagnosed until several years later when additional symptoms may develop. These include:

  • Pain and bloating in the abdomen
  • Diarrhea
  • Fatty foul-smelling stools
  • Bleeding from the intestines
  • Weight loss
  • Weakness
  • Fatigue (often from anemia )
  • Cough and chest pain
  • Increased skin pigmentation (in 50% of cases)
  • Swollen lymph glands

If the disease is not treated, this second stage may last for a number of years. Then more severe symptoms may occur due to the lack of nutrients reaching different parts of the body. Symptoms may occur in any part of the body, but most likely the heart, nervous system, brain, lungs, eyes, or skin. If the disease still is not treated, it usually causes death in this third stage.

Diagnosis

The doctor will ask about your symptoms and medical history, and perform a physical exam. Tests may include:

  • Blood and stool tests—to look for problems with absorbing nutrients ( malabsorption syndrome )
  • CT scan —a type of x-ray that uses a computer to make pictures of the inside of the body
  • Polymerase chain reaction (PCR) test—done on spinal fluid and the tissue of affected organs, to look for the bacteria that cause the disease
  • Biopsy —removal of a sample of tissue from your small intestine and other involved organs for testing

Treatment

Taking antibiotics can cure Whipple's disease. These drugs kill the bacteria that cause the disease. Your doctor will work with you to find the best types and doses for you. Often, a combination of antibiotics is used. The most commonly used antibiotics include:

  • Intravenous:
    • Ceftriaxone
    • Meropenem
    • Penicillin plus streptomycin
  • Oral:
    • TMP-SMZ (Bactrim or Septra)
    • Tetracyclines

If you have a more severe case of Whipple’s disease, you may also need intravenous antibiotics, fluid, and electrolyte replacement when you begin treatment. Electrolytes are salts and other substances in your body fluid that your heart and brain need to work properly. You may also need to take supplements of certain vitamins and minerals that your body is not absorbing normally on its own. These may include:

  • Iron
  • Folate
  • Vitamins A, B12, D, E, and K
  • Calcium
  • Magnesium

Symptoms usually go away after a few weeks to a few months on antibiotics. Your doctor may check your progress with the PCR test to see if any of the disease-causing bacteria are left. However, you will still need to take antibiotics for 1 to 2 years to prevent relapse of the disease.

Prevention

Currently, there is no known way to prevent Whipple’s disease.