For more than a century it has been known about Whipple's disease, but the infectious agent that causes it has not been sufficiently studied. Numerous systemic manifestations of the disease dictate the need for complex complex diagnostics, the final stage of which is a biopsy of the tissues of the affected small intestine. This ailment requires a long-term complex treatment with antibacterial, enzymatic preparations, vitamins and diet. Let us consider the indicated moments in more detail.
- 1 disease characteristics Whipple
- 2 reasons
- 3 three symptoms groups Whipple
- 3.1 extraintestinal manifestations
- 3.2 Intestinal symptoms
- 3.3 Systemic symptoms
- 4 Diagnostics
- 5 Complex treatment
- 5.1 The use of drugs
- 5.2 Therapeutic diet
- 5.2.1 Photogallery: features of the dietary intake of Whipple's disease
- 6 Possible complications of
Characteristics of Whipple's disease
Whipple's disease belongs to the group of Infectional diseases. In this pathological process, the defeat of several systems of the human body is developing.
. First of all, the intestine( small intestine) and nearby lymph nodes are affected. Then, during the development of pathology, other systems are involved: cardiovascular, nervous, musculoskeletal.
This is a fairly rare disease: according to statistics, it is found only in one person in a million. For the first time this syndrome was described in 1907, named after the author, the American doctor-pathologist Whipple George Hoyt.
The disease can occur at any age, but more often affects people in the middle age group from 40 to 55 years. Women are sick five times less often than men.
This disease belongs to the group of hereditary .
The causative agent of Whipple's disease is the little-studied bacterium Tropheryma whippelii. Some researchers believe that not only the presence of the infectious agent itself plays a role, but also other predisposing factors. These include:
- weakened body immunity, for example, after a long illness,
- persistent immunodeficiency of macrophages( T-cell), which leads to the inability of the body to resist bacteria and their life products.
Three groups of symptoms of Whipple's disease
This is a systemic disease, so its manifestations are very diverse. They can be divided into several groups corresponding to the phases of the development of the disease:
- Extraintestinal symptoms.
- Intestinal manifestations.
- Systemic manifestations( defeat of the musculoskeletal system, nervous, respiratory, genitourinary and cardiovascular systems of the body, as well as the eyes).
Extraintestinal manifestations of
It is believed that the disease begins precisely with extraintestinal symptoms, and pathology and bowel dysfunction develops later .Of the characteristic features are noted:
- fever( rise in body temperature to subfebrile, and sometimes febrile digits - 37-39 degrees);
- lesions of lymph nodes( located in the abdominal cavity, axillary and other groups);
- migratory joint pain, constantly changing its localization( pain occurs in one or the other joint);
- swelling and swelling of the affected joints, tenderness in palpation;
- pains and seals in muscles( and pain sensations appear not only during movements, but also at rest);
- moist cough with a detachable mucous character;
- pain in the chest.
The last two signs indicate the occurrence of lung lesions in a patient.
Intestinal symptoms of
They appear later and are associated with the development of bowel dysfunction:
- frequent light stool( diarrhea), can be up to 10-15 times a day, characterized by an unpleasant odor, with a high content of undigested fats, which is manifested by a greasy plaque;
- increased gassing or bloating( flatulence);
- abdominal pain, which is worse after eating;
- infringement of absorption of useful substances in a thin department of an intestine( the so-called malabsorption syndrome develops);
- the result of the above symptoms is a pronounced weight loss in the patient( and in the absence of necessary therapy this symptom progresses), as well as hypovitaminosis and protein deficiency.
In the process of disease progression, patients have symptoms that indicate an organism joining the pathological process of other organs and systems. The clinical picture may include a number of signs:
- disorders of the adrenal glands cause the appearance of areas of hyperpigmentation on the face;
- involvement in the pathological process of the heart leads to the development of valve pathologies, as well as to the onset of inflammation of the inner cardiac shell( endocarditis);
- from the lungs can develop pleurisy( accumulation of fluid in the pleural cavity);
- lesions of the nervous system are manifested by dementia, speech and sleep disorders, memory loss, depressive states;
- in patients may experience periodic seizures, and in severe cases - paralysis of the limbs;
- Vitamin A deficiency leads to the appearance of "night blindness", that is, significant deterioration of vision in the dark, at dusk or in poor light.
Because the disease manifests itself in the defeat of several body systems, the diagnosis of Whipple's disease should be comprehensive and include, in addition to examination, both laboratory and instrumental methods of investigation.
Biopsy of the tissues of the small intestine plays an important role in establishing the correct diagnosis. Pieces of the mucous are taken from the postbulbar region of the intestine during fibroadastroduodenoscopy( endoscopic examination of the stomach and the initial sections of the small intestine).Whipple's disease is established on the basis of the result of a histological examination of the biopsy specimen.
. From laboratory tests, it is prescribed:
- . A blood test is common( anemia and a decrease in red blood cell content can be detected).
- Blood analysis biochemical( able to establish lesions of internal organs: pancreas, liver).
- Stool analysis( or coprogram) allows you to identify fatty inclusions, remnants of undigested food and so on.
- The study of saliva( PCR) for the detection of a pathogen.
Instrumental methods that can be used to diagnose this pathological condition include:
- Ultrasound scan of the abdominal cavity and retroperitoneal space( for detection of internal organ damage).
- Echography of the heart( with suspicion of pathological changes in the valves, the inner shell of the heart).
- Computer or magnetic resonance imaging of the organs of the stomach, kidneys and adrenals, joints and stuff.
- X-ray examination of the intestine( most often performed with contrast - an aqueous suspension of barium sulfate, which the patient drinks before the procedure).
A competent doctor will necessarily conduct a thorough interview of the patient: in detail he will ask about complaints, the history of the appearance of unpleasant symptoms, the presence of diseases and pathologies of the digestive organs in the patient's relatives.
Complex treatment of
The use of medicines
The infectious nature of the disease determines the fact that the use of antibiotics always lies at the heart of the treatment regimen, most often preparations of the tetradycline .
When a progressive violation of the absorption and digestion of food is detected, enzymes are prescribed, such as Pancreatin, Pansinorm, Mezim-forte.
Also with therapeutic purpose are used:
- hormonal preparations( for anti-inflammatory effect);
- vitamins( groups A, D, E, K, B12, folic acid);
- iron preparations( with iron deficiency anemia established in the patient);
- triglycerides( Portalalak, fatty epithelium) in order to replace the lack of fat.
The prognosis of recovery with proper treatment is positive. In this case, therapy usually takes at least two years.
The patient is required to undergo a control examination of saliva for detecting the infectious agent of the disease( PCR method) in the middle and the end of the treatment course.
Patients suffering from Whipple's disease are recommended to have a treatment table number 5. According to this scheme of dietary nutrition, a fresh baking should be excluded from the diet:
- fat and fatty meat;
- fatty fish;
- sorrel and spinach;
- pepper, mustard;
- semi-finished products;
- fried meat;
- black coffee;
- ice cream.
The menu is recommended to be made up of the following products:
- wheat bread;Friable cereal grains
- soups on vegetable broth;
- lean meat( chicken, beef);
- low-fat cottage cheese;
- fruit( not acidic);
- tea( not strong);
Food should be consumed fractional, often( five to six times a day).
Photo gallery: features of the diet of the Whipple disease
are recommended Possible complications
If you do not seek medical help on time and do not perform treatment, the disease will progress with time and when the intestinal symptoms appear, the patient may dieafter few years. The main causes of death in Whipple's disease are:
- affection of the inner shell of the heart( endocarditis develops);
- pathological changes in the heart muscle( myocarditis);
- pathological accumulation of fluid in the pericardial cavity( pericarditis);
- edema of the brain( manifested by excessive fluid in the brain cells);
- adrenal insufficiency( adrenal function is impaired);
- iron deficiency anemia;
- cachexia( marked exhaustion of the body);
- multiple organ failure( manifests itself in the defeat of normal functioning of many organs and systems);
- recurrence of the disease( as a rule, this is noted with incorrectly selected treatment or failure to follow the doctor's recommendations).
If in the course of a person's illness irreversible changes in the body have developed, for example, polyarthritis, heart damage, expressed digestive disorders, then the question arises about disability( this must be addressed individually with the doctor in each case).
It can be said that the nearest forecast for the patient in most cases is favorable. It is more difficult to estimate the long-term prognosis, it depends on the frequency of relapses and possible complications, and also on whether the patient adheres to the proper treatment regimen.
Whipple's disease is characterized by systemic manifestations( intestinal and extraintestinal), which can lead to serious pathologies of the heart, adrenals, nervous system. In the absence of proper treatment, the patient may die within two to three years after the development of bowel dysfunction. However, the prognosis of recovery is positive. Prevention of the disease consists in preventing relapses, and for this it is necessary to visit the gastroenterologist on a regular basis, undergo a PCR examination to identify the pathogen. Also, you should strictly follow the recommendations of the gastroenterologist and adhere to a special diet.