Whipple disease is a rare infectious bacterial disease. This intestinal disease can cause multiple infections in different organs, and it can be fatal if left untreated.

Treatment with antibiotics is possible, but some patients relapse and need long-term treatment.

It is named after George Whipple, who discovered the bacteria in 1907.

Men are more susceptible than women, and 87 percent of people with Whipple disease are men aged between 40 and 60 years.

In the United States, Whipple disease affects fewer than one in every million people each year. In developing countries, it is more prominent, especially among children who live in areas where the sanitation system is poor.

However, it is more common elsewhere. In Senegal, In 2011, it was reported that nearly 44 percent of children aged between 2 and 10 years had the bacteria in their stools.

[Whipple up close - please credit Wikimedia user Mrwick1]Share on Pinterest
Whipple disease infects the lower intestine
Image credit: Wikimedia user Mrwick1 - own work.

Symptoms of Whipple vary widely among patients. The most common signs of the disease are weight loss and diarrhea.

Malabsorption can lead to weight loss, because the body cannot absorb nutrients through the bloodstream.

Other symptoms include:

  • stomach pain
  • weakness
  • fatigue
  • anemia
  • a darkening or graying of skin exposed to the sun

Endocarditis has been reported in a small number of cases. Sometimes it is the only symptom of Whipple disease.

Symptoms of endocarditis include breathlessness and swelling of the legs due to fluid buildup. The heart becomes inflamed and cannot pump fluid through the body.

Whipple disease can progress to the central nervous system (CNS).

If this occurs, a person begins to experience:

  • insomnia
  • vision issues such as uveitis
  • hearing loss
  • dementia
  • memory problems and changes in personality
  • facial numbness
  • loss of mobility and difficulty walking
  • various eye problems may occur, such as uveitis

The disease at this stage can become fatal.

Causes

A bacterial organism called Tropheryma Whipplei (T. Whipplei) causes Whipple disease by severely infecting the lining of the small intestine.

This infection can then spread to the heart, lung, brain, joints, and eyes. Whipple can affect any system of the body but presents most often in the small intestine.

The disease creates lesions on the wall of the small intestine and thickens the tissue. The tiny, finger-like protrusions from the wall that help absorb nutrients, called villi, are destroyed.

Individuals most likely to contract the disease are those with decreased ability to break down proteins and particles.

A bacterial organism called Tropheryma Whipplei (T. Whipplei) causes Whipple disease by severely infecting the lining of the small intestine.

This infection can then spread to the heart, lung, brain, joints, and eyes. Whipple can affect any system of the body but presents most often in the small intestine.

The disease creates lesions on the wall of the small intestine and thickens the tissue. The tiny, finger-like protrusions from the wall that help absorb nutrients, called villi, are destroyed.

Individuals most likely to contract the disease are those with decreased ability to break down proteins and particles.

Diagnosis of the bacteria that causes Whipple was not possible until relatively recently.

T. Whipplei was successfully recreated under laboratory conditions in 2000. This led to the development of a simple blood test to diagnose the disease.

The appearance of Whipple disease's characteristic intestinal lesions also assists in its diagnosis.

The best way to detect these lesions is to use an upper endoscopy to carry out a biopsy in the small intestine. During the procedure, a thin, flexible tube is passed through the mouth into the esophagus.

Endoscopic findings of Whipple disease include areas of thickened folds with a granular, yellow-white shaggy covering.

The polymerase chain reaction (PCR) method has been used to highlight DNA unique to T. Whipplei in various tissue samples of patients with Whipple disease.

The PCR method can confirm a diagnosis of Whipple disease when the diagnosis cannot be confirmed histologically, or by looking at the tissue through a microscope. PCR is highly sensitive and specific and is useful in suspicious, inconclusive cases.

In 1997, researchers suggested that a negative PCR result after therapy may predict a low clinical relapse rate. Patients who received treatment but still had a positive PCR result after therapy may be more likely to face a recurrence, they said.

Up to 50 percent of patients with Whipple disease have central nervous system infection, but not all of them develop clinical or radiologic evidence of the disease.

In these cases, it may be helpful to carry out a PCR analysis of the cerebrospinal fluid. This may help detect those with cerebral Whipple disease.

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Antibiotics are primary treatment for Whipple disease.

For many years, Whipple disease was considered a fatal primary metabolic disorder, but current treatment greatly improves a patient's chances of survival.

Antibiotics now are the primary treatment for Whipple disease. They can prevent symptoms from being fatal. Therapy may include penicillin with or without other antibiotics such as streptomycin or erythromycin.

Relapses are common. They can occur months or even years after initial treatment. They tend to affect the central nervous system, so any prescribed antibiotics need to have adequate blood-brain barrier penetration.

With effective treatment, the mucus membrane around the small intestine, referred to as the mucosa, reverts to normal. Bacteria disappear within a few days, and after 1 to 2 months, only dying organisms may be seen.

The structure of the finger-like villi also returns to its normal state. For some patients, however, it may take years for the intestine to return to normal.

For a good outcome and successful management of symptoms, the following factors are also important:

  • Proper fluid and electrolyte replacement is vital in patients with intestinal malabsorption.
  • Iron or folate supplements can help correct anemia if it occurs.
  • Vitamin D, calcium, and magnesium may be necessary to balance calcium levels.
  • Vitamin K can correct coagulopathy, or the correct clotting of the blood.

The patient's diet should be appropriately high in calories, protein, and other vitamins as Whipple disease can lead to malnourishment.

In rare cases, a long-term artificial supply of nutrients, also known as hyperalimentation, is necessary.

Prevention

Until now, there is no known way to prevent Whipple disease.

However, practicing good hygiene, for example, regular hand-washing, can reduce the risk.