Whipple's disease: symptoms, diagnosis and treatment

Whipple's disease in medical science also has other names - mesenteric lipogranulomatosis, intestinal lipophagic granulomatosis, intestinal lipodystrophy, intestinal lipodystrophy - a very rare systemic disease in which the area of ​​the small intestine is most often affected.

As it develops, this pathological process can affect the organs of the digestive, cardiovascular and nervous systems.

Who is the causative agent?

The causative agent of this rare disease is the currently unknown 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 translates into fever, stool disorders, productive coughing, seizures, and decreased visual acuity. Diagnostic methods for Whipple's disease must necessarily have an integrated approach. Laboratory research is necessary to identify a pathological pathogenic agent, and instrumental techniques to identify the degree of damage to internal organs.

The treatment of this pathology is, as a rule, conservative in nature and consists in the use of medications and a special diet. It should be noted that the treatment of the disease takes a very long period of time and is approximately two years.

More details of the symptoms and treatment of Whipple's disease are discussed below.

Etiology of the disease

Modern medical research has established that a pathological condition develops due to the penetration of certain pathogens into the human body, 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 healthy people, such cellular systems perform the following function - they absorb and destroy infectious agents, however, with 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 basic nutrients. This phenomenon is due to the fact that their transportation stops at the level of the intestine, and fat deposits are formed in the membranes - the so-called lipodystrophy.

Malabsorption

This situation is further exacerbated by violations in the absorption 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 also found that in a certain group of healthy people, in the absence of the main signs of the disease, a similar bacterium can be excreted along with saliva. In addition to the infectious cause, when a disease occurs, the reaction of the body itself plays an important role.

Predisposing factors

The main predisposing factors can be considered:

1. Decreased immunity.
2. Chronic pathology of the digestive tract.
3. Helminthic infestations.
4. Lack of complete nutrients from food.

It should be noted that the main risk category is people aged 45–55 years, but the likelihood of illness in other age groups is not excluded. In addition, it is known that men more often suffer from this infectious disease.

Classification

As it develops, Whipple's disease overcomes several stages, which develop gradually, one after another:

1. In the first stage extraintestinal symptoms develop. In this case, only one organ or system is often affected, for example, lymph nodes or joints. The main symptom is fever over a long period.
2. At the second stage of the disease, there is an occurrence of disorders in the digestive processes and the occurrence of some complications associated with this, for example, a sharp loss in weight due to upset stool.
3. At the third stage of Whipple's disease, involvement in the pathological process of internal organs - the heart, lungs, nervous system, etc. is noted.

Such an infectious disease has only one form - chronic.

Whipple's disease symptoms

Despite the fact that this disease has an infectious nature of occurrence, there are currently no specific data on the duration of the incubation period.

The first clinical signs of Whipple's disease are:

1. A sharp increase in temperature indicators (up to 38 degrees and above).
2. Muscle and joint pain.
3. Pronounced chills.
4. Swelling and redness of the skin over the affected joints.
5. An increase in the size of the lymph nodes - their mobility persists, with palpation of pain is not observed.
   

Other symptoms

Gradually, the development of intestinal or intestinal symptoms of Whipple's disease begins:

1. Violation of the act of defecation, which is expressed in profuse diarrhea - the frequency of urges sometimes reaches ten times a day.
2. Foamy consistency of feces and its light brown shade - in some cases, feces begin to acquire a tarry consistency, which is a consequence of a violation of blood coagulation or the development of internal hemorrhage.
3. Progressive weight loss.
4. Cramping pain reactions localized in the navel and often occurring after meals.
5. Nausea, in some cases provoking vomiting.
6. Feeling aversion to food.
7. Swelling and inflammatory lesions of the tongue.
8. The increase in the abdomen in size.
9. Increase in gas formation.
10. Excessive fatigue.

Changes in the skin

Some changes in the skin are also observed:

1. The appearance on the body of areas of hyperpigmentation.
2. Peeling and dry skin.
3. Sealing the skin.
4. Subcutaneous hemorrhage.

The following symptoms indicate involvement in the pathological infectious process of the lungs:

1. Severe cough with the formation of a large amount of sputum.
2. Pain in the chest area.
3. Dyspnea.
4. Persistent decrease in blood tone.
5. A slight increase in temperature.

Due to the development of the disease, damage to the nervous system may occur, which is expressed by the following phenomena:

1. Dementia.
2. Paralysis of the lower or upper extremities.
3. Convulsive seizures.
4. Disorders of speech functions.
5. Depressive conditions.
6. Sleep disturbance.
7. Impaired memory.

In some cases, the organs of vision may suffer:

1. Inflammatory lesions of the membranes of the eyes.
2. "Chicken" or night blindness.
3. Darkening of the skin around the eyes.

A similar clinical picture develops in both children and adults. It should be remembered that the severity of symptoms of Whipple's disease in children may be slightly higher than in older and middle-aged people.

Pathology diagnostics

Only a clinician who is familiar with the results of a wide range of instrumental and laboratory examinations is able to establish a diagnosis. If specific symptoms appear that are characteristic of a particular type of infectious disease, you should consult a physician who must perform a number of certain manipulations without fail. These include:

1. The study of the medical history, as well as a medical history.
2. Assessment of the condition of the skin and organs of vision.
3. Determination of temperature, blood tone and heart rate.
4. A detailed survey to establish the first time of the onset of the disease and the severity of the clinical picture.
5. Listening to the patient with a phonendoscope.

The next stage of diagnosis

The next step in the diagnosis of Whipple's disease will be laboratory diagnosis, which includes such tests:

1. General clinical and biochemical blood tests.
2. Hemogram.
3. PCR tests.
4. Coprogram.
5. Analysis of feces for occult blood and helminth eggs.
6. Histological studies of biopsy.

If a disease occurs, some instrumental methods are also used for diagnosis, the list of which includes:

1. Radiography of the peritoneum.
2. Ultrasonography of organs located in the abdominal cavity.
3. FGDS.
4. Irrigoscopy.
5. CT or MRI.
6. Biopsy.
7. ECG and echocardiography.

As an auxiliary diagnostic measures, in order to clarify the diagnosis, it is also necessary to consult the following specialists of a narrow profile:

• neurologist;
• cardiologist;
• gastroenterologist;
• rheumatologist;
• pediatrician - in the event of a pathological infectious process in a child.

What are the recommendations for Whipple's disease?

Disease treatment

Therapy of this infectious disease takes a very long time period and involves the use of exclusively traditional therapeutic methods. Drug therapy involves the following medicines:

1. Anti-inflammatory hormonal drugs.
2. Enzymatic agents.
3. Antibacterial medications.
4. General strengthening complex medicines.
5. Medications to improve the absorption of nutrients.
6. Medications of magnesium, potassium, calcium and iron.
7. Mineral and vitamin complex products.

During the treatment of Whipple's disease, it is extremely important for patients to follow a high-calorie diet, which assumes a high protein content. This means that the following products must be included in the diet:

• dairy products;
• seafood;
• legumes;
• low-fat varieties of fish and meat;
• wheat bread;
• soy and various cereal cereals;
• stewed fruit and weak green tea;
• vegetable soups;
• greens and salads;
• vegetables and fruits prepared by steaming or baking.

The list of categorically prohibited products includes:

• fresh muffin;
• a variety of confectionery;
• spinach and sorrel;
• semi-finished products;
• various cooking fats;
• offal;
• spicy and fatty dishes;
• ice cream and sweets;
• coffee and spirits.
• spices and all kinds of hot sauces.

Fractional and frequent food intake is recommended for patients with Whipple's disease. All the basic rules regarding the diet, the sample menu, as well as the list of allowed and prohibited products are provided by the attending physician.

Possible complications of the disease

In the complete absence of medical measures, the disease can cause deadly complications, such as:

1. Cerebral edema.
2. Anemia (anemia).
3. Adrenal insufficiency.
4. Serious myocardial damage.
5. Loss of patient ability to work.
6. Extreme degree of exhaustion.
7. Multiple organ failure.
8. Frequent exacerbations.

Prevention and prognosis

In order to reduce the risk of this disease, certain simple rules should be followed. Preventive recommendations should include:

• a complete rejection of harmful addictions - alcohol, smoking;
• rational and quality nutrition;
• strengthening immunity;
• complex treatment of various gastroenterological and other pathologies occurring in chronic forms that can provoke the onset of the disease;
• regular full medical examination.

Symptoms 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, adherence to certain therapeutic rules helps to achieve long periods of remission.

Clinical guidelines for Whipple's disease should be strictly followed.