Whipple’s disease or intestinal lipodystrophy

Whipple’s disease (EW) or intestinal lipodystrophy is a disease produced by the bacteria Tropheryma whipplei. Although what is most known is intestinal involvement, it is a mulsystemic pathology that affects many organs such as the nervous system and joints.

Is a rare disease that mainly affects males About 40 years old. The cases published do not reach the 2000 affected.

Pathogenesis of Whipple’s disease

It is not known exactly how the bacteria reaches our body. Since the main manifestations are intestetinales, it is believed that enters our body orally, by drinking water or other foods that contain the microorganism.

Tropheryma whipplei It is a commensal microorganism, that is, it does not produce a disease by itself. In fact, up to a third of the population carries this bacterium. It can remain in the body for years without producing clinical symptoms.

After a more or less long asymptomatic period, in some subjects the disease appears. Certain factors break the balance of our body with the bacteria and it becomes harmful to the organs.

The causes that determine who has the disease and who does not are known. Genetic theories have been exposed since it is much more common in males, but much remains to be discovered.

Within our organism, the bacterium Tropheryma whipplei is divided into cells of the immune system called macrophages . These cells are responsible for capturing bacteria and destroying them. It seems that the macrophages of patients with EW capture the bacteria but can not destroy it. Therefore, it is divided producing the death of macrophages.

Whipple’s disease clinic

Whipple's disease or intestinal lipodystrophy

Whipple’s disease It is characterized by a triad of symptoms :

  • Joint pains or arthralgias that may appear years before the rest of symptoms.
  • Weightloss that can reach 20 kg.
  • Diarrhea.

It goes through different stages of evolution. The onset of the disease is very unspecific with fever, joint pain, loss of appetite, fever, abdominal pain, etc.

Joint pain is usually multiple, affecting several joints. It can affect anyone, although it is more common to appear in the joints of the legs. It is common to distribute symmetrically, that is, to affect the same joint on both sides of the body.

As the picture progresses highlights intermittent diarrhea associated or not with other intestinal symptoms. In addition, diarrhea is accompanied by symptoms of malabsorption of nutrients, such as the presence of fat in the stool (steatorrhea, stool floating in the toilet) or loss of muscle mass.

When the disease has been established other symptoms appear such as weight loss, neurological alterations and skin alterations.

Neurological involvement usually produces dementia, so you may have interest in the differential diagnosis of other similar tables such as Alzheimer disease . On the other hand, skin involvement usually manifests with areas of darker skin (hyperpigmented) in areas exposed to the sun.

Diagnosis of Whipple’s disease

Whipple's disease or intestinal lipodystrophy

The suspicion of this pathology is made by the clinic. A blood test can be done, although it does not confirm the disease. Some of the findings we can find are:

  • Signs of inflammation in early stages: increased leukocytes, anemia or increased platelets. The acute phase reactants are also usually elevated. It is called this way to different molecules of the organism whose levels rise if there inflammation .
  • Malabsorption data, when diarrhea has been established.

To confirm Whipple’s disease you have to prove the existence of Tropheryma whipplei in the body .

To do this you have to perform a biopsy, Normally intestinal. What is done is to extract a portion of the intestine and analyze it microscopically. Macrophages will be observed inside which are vacuoles or granules that are stained with a material called PAS. They are said to be PAS + vacuoles.

When the clinic is not so clear other tests can be performed as an NMR cranial nerve in the case of neurological involvement to rule out other causes of dementia.

If on the contrary, the suspicion of EW is high and the biopsy does not show specific data, the DNA of T. whipplei. This is achieved through molecular tests, such as the polymerase chain reaction or PCR.

Treatment of Whipple’s disease

Whipple's disease or intestinal lipodystrophy

Once the diagnosis is confirmed, to end the infection you have to use antibiotics for a long period of time . Drugs that reach the nervous system must be used to prevent the disease from reactivating. The cotrimoxazole at least for 1 year.

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