Whipple's disease or intestinal lipodystrophy - a systemic disease of infectious nature, characterized by lesions of the small intestine with typical infiltration of the intestinal mucosa "foamy macrophages", Gram-positive bacillus similar little bodies. Also, there is the accumulation of lipids in it, the expansion of the lymphatic vessels, the development of malabsorption syndrome. Whipple's disease develops predominantly in men (30: 1) age group 30 - 50 years. According to various studies annually registered about 30 cases in the world.
The etiology of Whipple's disease
Whipple's disease was first described in 1907 by a pathologist J.
. Whipple that the autopsy found an increase in mesenteric lymph nodes poliserozita
. Bacterial infection was confirmed only in 1992
. Using polymerase chain reaction were identified Tropheryna whipelii - gram-positive actinomycetes
. Actinomycetes are detected in the active stage of the disease in the mucosa of the small intestine
. The primary cause of the disease is now anticipated disruption of the functioning of the immune system of different origin
. For the active stage of the disease characterized by localization of bacteria in macrophages, indicating the loss of their ability to lysis of microorganisms
. With the development of Whipple's disease is observed decreased production of antibodies to the pathogen in the body, or the complete lack thereof
. It becomes more pronounced lack of cellular immune response
Despite the fact that the pathogen has been identified, the causes of Whipple's disease is still unclear. Scientists do not exclude a genetic predisposition. A significant pathogen contamination of waste water and soil does not lead to a massive infection. So far, not a single recorded case of Whipple's disease transmission from person to person. Researchers believe that not all strains of the pathogen may develop Whipple's disease and its characteristic pathological changes.
The body's response to the introduction of the pathogen of Whipple's disease are pathological changes in organs and systems. Thus, Whipple's disease primarily occurs intestinal lesion. In view of dissemination of the pathogen Tropheryna whipelii most patients observed defeat of the musculoskeletal system, central nervous system, cardiovascular system, respiratory system.
Of particular importance in the development of Whipple's disease is a defeat of the small intestine and the local lymphatic system. The intestinal mucosa infiltrated microphages. There lymphorrhoid, fat accumulation in the intercellular space of the small intestinal mucosa.
Structural bowel disorders lead to the development of malabsorption, which hinders the transport of nutrients.
Stage of the disease. Symptoms of Whipple's disease
During the Whipple's disease are three stages:
- The first stage is characterized by the manifestation of extra-intestinal symptoms. At this stage, the main symptoms of the disease are arthritis, fever of unknown origin, muscle aches, cough with sputum. The first stage of the disease can last from 3 to 8 years;
- The second stage of the disease is characterized by the manifestation of gastrointestinal symptoms. The main symptoms of Whipple's disease is the second stage of chronic diarrhea with copious stools up to 10 times a day, abdominal pain, pain on palpation (possible to detect enlarged lymph nodes), loss of appetite, weight loss, muscle atrophy and weakness. The most significant changes at Whipple's disease are disorders of lipid and carbohydrate metabolism, polyhypovitaminosis, hypocalcemia, the development of severe malabsorption syndrome;
- The third stage of Whipple's disease is characterized by the development vnegastrointestinalnyh symptoms, systemic manifestations. For the third stage of the disease characterized by dysfunction of the adrenal glands (low blood pressure, nausea, vomiting, anorexia, skin pigmentation), signs of central nervous system (disorder of hearing and vision, the defeat of the cranial nerves), the defeat of the cardiovascular system (endocarditis, pericarditis, myocarditis) , skin changes.
Diagnosis of Whipple's disease
A variety of clinical changes in the target organism difficult to diagnose Whipple's disease. The main method of diagnosis is the analysis of biopsy (excision of the mucous membrane of the upper wall of the intestine), observation of morphological changes in the intestinal endoscopy. The most accurate method is PCR diagnostics (polymerase chain reaction) that allows identification of the agent of Whipple's disease, as a biopsy or other biological fluids.
The treatment of Whipple's disease
For a long time it was believed that Whipple's disease is characterized by death. The use of intensive antibiotic therapy does more favorable prognosis. The basis of treatment Whipple's disease different antibiotics are used. Most effective among them are drugs tetracycline. The dosage and timing of therapy is determined on the basis of histological examination in each case individually.
In severe forms of the disease patients often require additional symptomatic treatment.
To eliminate symptoms of malabsorption patient is assigned a special diet, rich in animal protein with limited fat (up to 30 grams per day). To eliminate the symptoms of diarrhea and abdominal discomfort patients are assigned spazmolitki delaying intestinal peristalsis.
The effectiveness of applied therapy is assessed by histological examination of the repeat biopsy. The main feature of the effectiveness of treatment is reduction in the number of macrophages in the sample or a complete lack thereof. Symptoms of Whipple's disease may manifest a bit longer due to the scale of the defeat.