Whipple's disease

Whipple's disease or intestinal lipodystrophy – the general disease of the infectious nature which is characterized by damage of a small intestine with typical infiltration of a mucous membrane of intestines "foamy macrophages", gram-positive batsillopodobny little bodies. Also accumulation in it of lipids, expansion of absorbent vessels, development of a sprue is observed. Whipple's disease develops preferential at men (30:1) age categories of 30 - 50 years. According to various researches about 30 cases in the world annually are registered.

Etiology of a disease of Whipple

For the first time Whipple's disease was described in 1907 by the pathologist J. Whipple who when opening found increase in mezenteralny lymph nodes with a polyserositis. The bacterial nature of a disease was confirmed only in 1992. By means of polymerase chain reaction Tropheryna whipelii – gram-positive actinomycetes were identified. Actinomycetes are found in an active stage of a disease in mucous membranes of a small intestine. The main reason for development of a disease supposes disturbance of functioning of immune system of various origin now. Localization of bacteria in microphages is characteristic of an active stage of a disease that indicates abilities to a lysis of microorganisms their loss. At a course of a disease of Whipple reduced development of antibodies to the activator in an organism or its total absence is observed. More expressed there is a lack of a cellular immune response.

In spite of the fact that the infestant was identified, the reasons of a course of a disease of Whipple still remain not clear. Scientists do not exclude genetic predisposition. Considerable contamination by the activator of sewage and soils does not lead to mass infection. So far no uniform case of transfer of a disease of Whipple from the person to the person is registered. Researchers believe that not all strains of the activator can develop Whipple's disease and pathological changes, characteristic of it.

Response of an organism to implementation of a disease-producing factor of Whipple are pathological changes in bodies and their systems. So, at Whipple's disease first of all there is a damage of a small intestine. In view of dissimination of the Tropheryna whipelii activator at most of patients defeat of a musculoskeletal system, the central nervous system, cardiovascular system, bronchopulmonary system is observed.

Special value in a course of a disease of Whipple has damage of a small intestine and local lymphatic system. Mucous membrane of intestines of an infiltrovan microphages. Expansion of absorbent vessels, accumulation of fat in intercellular space of a mucous membrane of a small intestine is observed.

Structural disturbances of intestines lead to malabsorption development that complicates transport of nutrients.

Disease stages. Symptoms of a disease of Whipple

During Whipple's disease distinguish three stages:

• The first stage which is characterized by manifestation of abenteric symptoms. At this stage of a disease of the main symptoms polyarthritis, fever of not clear origin, muscle pain, cough with expectoration are. The first stage of a disease can last from 3 to 8 years;
• The second stage of a disease is characterized by manifestation of gastrointestinal symptoms. The main symptoms of a disease of Whipple of the second stage are chronic diarrhea with a plentiful chair to 10 times a day, abdominal pain, painful feelings at a palpation (detection of the increased lymph nodes is possible), a loss of appetite, decrease in body weight, an atrophy of muscles and their weakness. The most serious changes at Whipple's disease are disturbances of fatty and carbohydrate exchanges, a polyhypovitaminosis, a hypocalcemia, development of a heavy sprue;
• The third stage of a disease of Whipple is characterized by development of extra gastrointestinal symptoms, system manifestations. The dysfunction of adrenal glands (lowered by the ABP, nausea, vomiting, anorexia, a xanthopathy), signs of defeat of TsNS (disorder of hearing and sight, defeat of cranial nerves), defeat of cardiovascular system (endocarditises, perikardita, myocardites), skin changes are characteristic of the third stage of a disease.

Diagnosis of a disease of Whipple

A variety of clinical changes in the struck organism complicates diagnosis of a disease of Whipple. The main diagnostic method is the analysis of a bioptat (the excised wall of a mucous membrane of upper parts of intestines), observation of morphological changes of intestines at endoscopy. The most exact is the method of PTsR-diagnosis (polymerase chain reaction) allowing to define a disease-producing factor of Whipple both in a bioptata, and in other biological environments.

Treatment of a disease of Whipple

Long time was necessary that the lethal outcome is characteristic of Whipple's disease. Use of intensive antibacterial care does the forecast more favorable. At the heart of treatment of a disease of Whipple various antibiotics are applied. The most effective among them are drugs of a tetracycline row. The dosage and terms of therapy are defined on the basis of histologic researches in each case individually.

At severe forms of a disease quite often patients need an additional symptomatic treatment.

For elimination of symptoms of malabsorption to the patient the special diet enriched with animal protein with the limited content of fats is appointed (to 30 g a day). For elimination of symptoms of diarrhea and abdominal discomfort to patients the spazmolitka who are slowing down an intestinal peristaltics are appointed.

Efficiency of the applied therapy is estimated by carrying out a repeated histologic research of a bioptat. Decrease in quantity of macrophages in the studied sample or their total absence is the main sign of efficiency of treatment. Symptoms of a disease of Whipple can be shown slightly longer owing to scale of defeat of bodies.