Whipple’s disease

Whipple’s disease

What is it ?

Whipple’s disease is an infectious disease. The bacteria causing the disease is Tropheryma whipplei (T. whipplei), capable of invading the whole organism.

During infection with this pathogen, certain clinical manifestations are associated but not obligatory. These are weight loss, polyarthritis, diarrhea, fever, lymphadenopathy (infection affecting the lymph node), valve disease (disease of the heart valves), endocarditis (infection of the inner membrane of the heart: l endocardium), pleuritis (inflammation of the pleura, membrane covering the lungs), eye diseases or tenosynovitis (inflammation of the tendons). (1)

In rarer cases, neurological damage may also be visible in Whipple’s disease.

The bacterium in question is very widely found in wastewater treatment plants and can be excreted in the stool.

In addition, scientists suspect an inherited cause in the development of the disease. (1)

It is a rare disease affecting the small intestine. This intestinal part is part of the upper surface of the gastrointestinal tract, from the stomach to the colon. Most food is therefore absorbed from this part of the digestive tract. The small intestine can be broken down into several parts: the duodenum, the jejunum and the ileum. After infection of this part of the body, the bacteria can spread throughout the body and thus cause significant damage. Among these organs and tissues, we can mention:

– ligaments;

– the central nervous system (brain, spinal cord, peripheral nerves);

– hearts ;

– the eyes ;

– lungs.

Without prompt management and adequate treatment in place, Whipple’s disease can be serious and require lifelong treatment.

Symptoms

The specific symptoms of Whipple’s disease can vary widely from patient to patient. The most common manifestations include:

– diarrhea;

– weight loss caused by intestinal malabsorption.

In some cases, the patient may not have diarrhea but have other symptoms such as:

– the presence of white and yellowish spots in the intestinal mucosa;

– joint pain with or without inflammation;

– fatty stools;

– cramps and bloating in the chest;

– enlargement of the lymphatic vessels;

– loss of appetite;

– fever ;

– intense fatigue;

– muscle weakness;

– darkening of the skin.

In more severe cases of the disease, affected individuals may also present with neurological symptoms such as:

– eye deficiencies;

– memory loss and personality changes;

– numbness in the face;

– headaches:

– difficulty in walking;

– hearing loss;

– some dementia.


Finally, slightly less common but possible symptoms can be significant of the disease:

– a persistent cough;

– pain in the chest;

– pericarditis, which is inflammation of the membrane surrounding the heart;

– heart failure no longer allowing the heart to pump blood correctly and thus distribute it throughout the body.

The origins of the disease

The origin of the development of Whipple’s disease is bacterial. The bacteria involved is Tropheryma whipplei (T. whipplei) infecting the upper part of the small intestine first and then spreading throughout the body in a second.

In the case of infection with this bacteria, the intestinal villi then take on an abnormal appearance and no longer absorb nutrients properly. In this sense, the most commonly associated symptoms are diarrhea and malnutrition.

The bacteria can infect any individual. Nevertheless, male Caucasian populations, aged between 40 and 60 years are more affected by the disease.

Whipple’s disease is still a rare disease, the prevalence of which (number of cases in a given population at a given time) amounts to 1 / 1 people. Some people are more subject to the development of the pathology, such as farmers, workers in wastewater treatment plants, etc.

The exact mode of transmission of the disease is not currently known, however, scientists are looking into the fact that the bacteria is widely found in soils and wastewater. In addition, the bacteria can be found both in healthy individuals or those with underlying disease. Inter-personal transmission has been shelved.

Finally, some researchers have demonstrated the development of the disease through the presence of certain genetic factors.

Risk factors

The risk factors associated with Whipple’s disease are:

– exposure to the bacteria Tropheryma whipplei, in particular at the level of agricultural land and wastewater treatment plants;

– certain genetic factors present within the family, predisposing the subject to the disease.

Prevention and treatment

The diagnosis of Whipple’s disease is made through various tests and examinations. Notably :

– family history;

– physical examinations: examination of the patient’s body, listening to breathing with a stethoscope, etc. ;

– blood tests allowing the verification of malabsorption in view of a reduction in the level of proteins, vitamins and calories in the blood. Also in view of the abnormal electrolyte level, anemia or the presence of DNA from T. whipplei .

– an endoscopy and / or an enteroscopy allowing to have an image of the inside of the intestine,

The general practitioner can make a first diagnosis, however, a consultation with a gastroenterologist is generally necessary in the validation of the diagnosis and in the management of the disease.

Treatment of the disease is based on antibiotic therapy to destroy the bacteria.

The prescription of antibiotics is usually intravenously during the first two weeks of treatment. This intravenous antibiotic therapy generally includes:

– the ceftriaxone;

– the meropenom;

– penicillin;

– streptomycin.

After this intravenous antibiotic therapy, the doctor usually prescribes oral antibiotic therapy for the long term (usually over 2 years). These antibiotics resume:

— la trimethoprim/sulfamethoxazole ;

– the doxocycline.

The treatment must be followed until the complete destruction of all the bacteria T. whipplei within the intestine and all other parts of the body. For this, close monitoring must be carried out by the doctor to ensure the total disappearance of bacterial strains. This monitoring is carried out through repeated blood tests or recurrent endoscopies.

In more severe cases of the disease, with neurological disorders, other drugs may be prescribed, such as:

– other antibiotics;

– hydrochloroquine, an anti-malaria drug;

– weekly injections of gamma interferons, allowing the activation of the immune system;

– corticosteroids, to limit inflammation.

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