Whipple’s disease – a rare infectious disease

Diarrhea and inadequate nutrient absorption (malabsorption) can have various causes. In rare cases it can be due to Whipple's disease. In addition to the small intestine, a general infection affects various other organ systems. It is believed that infection with the pathogen Tropheryma whipplei causes the systemic disease. Due to the damage to the small intestine, the disease leads to diarrhea and a disturbance in nutrient absorption. This can result in drastic weight loss or anemia due to iron deficiency. The pathogen was named after the disturbance in nutrient uptake: Tropheryma is Greek and consists of the two terms trophe (nutrition) and eryma (Cabinets) together. Even if the disease is very rare - it must be recognized and treated quickly. If left untreated, Whipple's disease takes a fatal course.

What causes Whipple's disease?

It is believed that Whipple's disease is caused by the pathogen Tropheryma whipplei. But infection is not the only cause. A defect in the cellular immune system also probably plays a role. In particular, the T cells (T lymphocytes) seem to have limited functionality. It has also been shown that the so-called phagocytes of the immune system (macrophages) do not produce certain signal substances (monokines) sufficiently in Whipple sufferers. This is, for example, the signal substance Interleukin-12. It is usually responsible for activating the T cells in the event of an infection and fighting an infection. Genetic predisposition could also contribute to the onset of the disease.

What is Tropheryma whipplei?

The pathogen Tropheryma whipplei is a rod-shaped bacterium of the actinomycete species. It is widespread in the environment. It can be found in sewage, among other things.

Is Whipple's disease contagious?

No, Whipple's disease is not contagious. Unfortunately, the exact route of transmission of the infection is still unknown. It is believed that the disease is caused by the pathogen Tropheryma whipplei. But infection does not seem to be the sole cause. Because the pathogen is found in the stool of two to four percent of the population - without any complaints. Therefore, experts suspect that a malfunction of the cellular immune system, more precisely the phagocytes (macrophages) and T cells play a role. Genetic predisposition could also contribute to the onset of the disease.

More about the immune system Our immune system is very complex, Many processes are still not clear. Since a disturbance in the immune system leads to the outbreak of Whipple's disease, a simplified explanation follows here. Many different cells and signal substances play a role in the fight against pathogens. The immune system is divided into two main systems: The innate, nonspecific immune system The acquired, specific immune system They belong to the latter T cells. These blood cells are formed in the bone marrow and receive a receptor in the thymus which, according to the key-lock principle, fits to very specific foreign substances (antigens). If a T cell is presented with a suitable foreign substance via a phagocyte (macrophage), for example, the T cell is activated. The T cell activates further T cells via messenger substances (cytokines). These can then destroy infected body cells and fight the infection. The infection can only be successfully combated if all the cells of the immune system and signaling substances work together.

What are the symptoms of Whipple's disease?

If you suffer from the symptoms below and all other common causes have been excluded, Whipple's disease should be considered. In this case, you should contact a specialized center.

The most important symptoms at a glance:

• Combination of chronic diarrhea, joint pain and weight loss
• Chronic joint inflammation (arthritis) without antibody detection (seronegative)
• Paralysis of the eyes and small muscle twitches (myoclonus)
• Inflammation of the inner lining of the heart (endocarditis) without detection of pathogens (culture negative)

Since Whipple's disease is a disease of the entire body (systemic disease), the symptoms are varied. Typical are:

• Joint pain
• Diarrhea
• Weight loss
• Fever
• Lymphadenopathy

However, joint pain (arthralgia) often occurs many years before indigestion. Depending on the organ affected, sufferers have further symptoms. For example, if the central nervous system is affected, neurological symptoms occur over a longer period of time. This table describes the symptoms for the individual organ systems. The symptoms described can occur individually or in combination. This is exactly what makes the infestation so insidious. In any case, Whipple's disease must be ruled out if these symptoms occur, even if it is a very rare disease.

How is Whipple's disease treated?

Whipple's disease is treated with appropriate high-dose antibiotic therapy. This must be done through the veins (intravenously) in the hospital for two weeks. This is followed by so-called maintenance therapy. Due to the severity of the disease, maintenance therapy with oral antibiotics must be carried out for one year! If antibiotic therapy is undertaken in good time, healing will occur.

Danger of death!

If left untreated, the disease is fatal!

Does Whipple disease also occur in women?

Yes, women can also be affected by the disease. It used to be thought that men were affected eight times more often. Recent studies tend to assume a 3:1 distribution. The main age of onset of Whipple's disease is between the ages of 40 and 55. The disease usually does not occur in people under 30 years of age.

Does Whipple's disease also appear in the kidney?

In principle, the disease can affect any organ. However, kidney involvement is rarely reported.

What should I eat if I have Whipple's disease?

There are no nutritional recommendations in the guidelines for Whipple's disease. Depending on the intestinal symptoms, individual nutritional advice should be given. Because Whipple's disease often leads to strong weight loss and malnutrition this must be taken into account. These symptoms need to be treated therapeutically. You are welcome to get information from our qualified nutritionists here. They will help you to make the right decisions under professional supervision. If you do have Whipple's disease, you need to act quickly and start antibiotic therapy immediately.