Whipple's disease in medical science has other names - mesenteric lipogranulomatosis, intestinal lipophagic granulomatosis, intestinal lipodystrophy, intestinal lipodystrophy - a very rare systemic disease in which the small intestine area is most often affected.
As it develops, this pathological process can affect the organs of the digestive, cardiovascular and nervous systems.
Who is the trigger?
The causative agent of this rare disease is the currently little-studied pathogenic bacterium Tropheryma whippelii, and the main factor provoking pathology in medicine is considered to be a decrease in the resistance of the immune defense in the body.
The clinical picture clearly reflects the symptoms of the affected organs or systems. Often this is expressed in fever, stool disorders, productive cough, convulsive seizures and decreased visual acuity. Diagnostic methods for Whipple's disease must necessarily have an integrated approach. At the same time, laboratory studies are necessary to identify a pathological pathogen, and instrumental techniques are necessary to identify the degree of damage to internal organs.
The treatment of this pathology is usually conservative and consists in the use of medications and adherence to a special diet. It should be noted that the therapy of the disease takes a very long period of time and is approximately two years.
For more on the symptoms and treatment of Whipple's disease, see below.
Etiology of disease
Modern medical research has established that a pathological condition develops as a result of the penetration into the human body of certain pathogens, which are Tropheryma whippelii. After infection, these bacteria begin to actively increase their numbers in the cells of the immune system, which are called macrophages.
In he althy people, such cellular systems perform the following function - they absorb and destroy infectious agents, however, in Whipple's disease, pathological microorganisms remain unharmed. Macrophages, the structure of which is changed under the influence of these bacteria, begin to accumulate in the mucous membranes of the small intestine, which, in turn, leads to a sharp decrease in the absorption of essential nutrients. This phenomenon is due to the fact that their transportation stops at the level of the intestine, and fat deposits form in the membranes - the so-called lipodystrophy.
Malabsorption
This situation is further exacerbated by malabsorption of minerals, vitamins and other nutrients. Subsequently, the pathological process begins to spread to the lymph nodes located inside the abdominal cavity, to the pericardium, myocardium, pleura, joints and brain.
What is the endoscopic picture of Whipple's disease?
Studies of the pathological process have also found that in a certain group of he althy people, in the absence of the main signs of the disease, such a bacterium can be excreted along with saliva. In addition to the infectious cause, the reaction of the body itself plays an important role when a disease occurs.
Predisposing factors
The main predisposing factors can be considered:
- Decreased immunity.
- Chronic pathologies of the digestive tract.
- Worm infestations.
- Lack of complete nutrients from food.
It should be noted that the main risk category is people aged 45–55 years, but the possibility of the disease in other age groups is not excluded. In addition, it is known that men are more likely to suffer from this infectious disease.
Classification
As it develops, Whipple's disease overcomes several stages that develop gradually, one after another:
- Extraintestinal symptoms develop in the first stage. In this case, only one organ or system is often affected, for example, lymph nodes or joints. Maina symptom is a high temperature over a long period.
- At the second stage of the disease, there are disturbances in the digestive processes and the occurrence of some complications associated with this, for example, a sharp weight loss due to stool disorder.
- In the third stage of Whipple's disease, there is involvement in the pathological process of internal organs - the heart, lungs, nervous system, etc.
Such an infectious disease has only one form - chronic.
Symptoms of Whipple's disease
Despite the fact that this disease has an infectious nature of occurrence, there are currently no specific data regarding the duration of the incubation period.
The first clinical signs of Whipple's disease are:
- A sharp increase in temperature indicators (up to 38 degrees and above).
- Muscle and joint pain.
- Severe chills.
- Swelling and redness of the skin over affected joints.
- Increase in the size of the lymph nodes - their mobility is preserved, no pain is observed on palpation.
Other signs
Intestinal or intestinal symptoms of Whipple's disease gradually begin to develop:
- Violation of the act of defecation, which is expressed in profuse diarrhea - the frequency of urges sometimes reaches ten times a day.
- Foamy consistency of stool and its light brown tint - in some cases, feces begin to becometarry consistency, which is a consequence of a violation of blood clotting or the development of internal hemorrhage.
- Progressive weight loss.
- Cramping pain reactions, localized in the navel and often occurring after meals.
- Nausea, in some cases provoking vomiting.
- Feeling disgusted with food.
- Swelling and inflammation of the tongue.
- Increased belly size.
- Increased gas production.
- Excessive fatigue.
Skin changes
There are also some changes in the skin:
- The appearance of areas of hyperpigmentation on the body.
- Skin peeling and dryness.
- Skin thickening.
- Subcutaneous hemorrhages.
The following symptoms indicate involvement in the pathological infectious process of the lungs:
- Violent cough with a lot of phlegm.
- Pain in the chest area.
- Shortness of breath.
- Persistent decrease in blood tone.
- Slight increase in temperature readings.
Due to the development of the disease, damage to the nervous system may occur, which is expressed by the following phenomena:
- Dementia.
- Paralysis of lower or upper limbs.
- Convulsive seizures.
- Disorders of speech functions.
- Depressive states.
- Sleep disturbance.
- Violation of memory.
Bin some cases, the organs of vision may suffer:
- Inflammatory lesions of the membranes of the eyes.
- "Chicken" or night blindness.
- Darkening of the skin around the eyes.
Such a clinical picture develops in both children and adults. It should be remembered that the severity of the symptoms of Whipple's disease in children may be slightly higher than in older and middle-aged people.
Diagnosis of pathology
Only a clinician who is familiar with the results of a wide range of instrumental and laboratory examinations can establish a diagnosis. If specific symptoms appear that are characteristic of a particular type of infectious disease, you should consult a therapist, who must necessarily perform a number of certain manipulations. These include:
- Studying the history of the disease, as well as taking an anamnesis.
- Assessment of the condition of the skin and organs of vision.
- Determination of temperature, blood tone and heart rate.
- Detailed survey to establish the first time of onset of the disease and the severity of the clinical picture.
- Listening to the patient with a phonendoscope.
Next diagnostic step
The next step in the diagnosis of Whipple's disease will be laboratory diagnostics, which includes the following tests:
- General clinical and biochemical blood tests.
- Hemogram.
- PCR tests.
- Coprogram.
- Feces analysis for occult blood and helminth eggs.
- Histological examination of the biopsy.
When a disease occurs, some instrumental methods are also used for diagnosis, the list of which includes:
- X-ray of the peritoneum.
- Ultrasonography of organs located in the abdominal cavity.
- FGDS.
- Irrigoscopy.
- CT or MRI.
- Biopsy.
- ECG and EchoCG.
As ancillary diagnostic measures to clarify the diagnosis, consultations of the following narrow specialists are also necessary:
- neurologist;
- cardiologist;
- gastroenterologist;
- rheumatologist;
- pediatrician - in the event of a pathological infectious process in a child.
What are the recommendations for Whipple's disease?
Cure disease
Therapy of this infectious disease takes a very long time period and involves the use of exclusively traditional therapeutic techniques. Drug therapy involves taking the following medications:
- Anti-inflammatory hormonal drugs.
- Enzymatic agents.
- Antibacterial drugs.
- General tonic complex medicines.
- Drugs to improve nutrient absorption.
- Medicines of magnesium, potassium, calcium and iron.
- Mineral-vitamin complex products.
During the treatment of Whipple's disease, it is extremely important for patients to follow a high-calorie, high-protein diet. This means that the following foods must be included in the diet without fail:
- dairy products;
- seafood;
- legumes;
- low-fat varieties of fish and meat;
- wheat bread;
- soy and various cereals;
- compotes and weak green tea;
- vegetable soups;
- greens and salads;
- vegetables and fruits cooked by steaming or baking.
The list of strictly prohibited products includes:
- fresh bun;
- variety of confectionery;
- spinach and sorrel;
- semi-finished products;
- various cooking oils;
- offal;
- spicy and fatty foods;
- ice cream and sweets;
- coffee and spirits.
- spices and all kinds of hot sauces.
Patients with Whipple's disease are advised to eat small and frequent meals. All the basic rules regarding the diet, an approximate menu, as well as a list of allowed and prohibited foods are provided by the attending physician.
Possible complications of the disease
In the complete absence of therapeutic measures, the disease can cause deadly complications, such as:
- Cerebral edema.
- Anemia (anemia).
- Insufficiencyadrenal.
- Serious myocardial damage.
- Patient Disability.
- Extremely exhausted.
- Multiple organ failure.
- Frequent exacerbations.
Prevention and prognosis
In order to reduce the risk of this disease, you should follow certain simple rules. Preventive advice should include:
- complete rejection of bad habits - alcohol, smoking;
- rational and quality nutrition;
- strengthening immunity;
- complex treatment of a variety of gastroenterological and other pathologies that occur in chronic forms that can provoke the onset of the disease;
- Regular full medical examination.
Symptomatology and therapy of pathology significantly affect the prognosis, which is conditionally considered favorable. This is because it is impossible to completely cure such a disease, however, compliance with certain therapeutic rules helps to achieve long periods of remission.
Clinical guidelines for Whipple's disease must be strictly followed.
