Systemic diseases have always been a mystery to the medical world. One of them is sarcoidosis, which does not have a valid etiology, but manifests itself in the form of inflammation of the connective tissue in various organs and tissues with the formation of granulomas. Lefgren's syndrome is a special course of sarcoidosis that affects the skin, intrathoracic lymph nodes, as well as joints.
Development reasons
The basis of pathogenesis is the development of an autoimmune reaction. In the immune system, a malfunction occurs, and white blood cells begin to affect healthy cells in the lungs, joints, and skin.
Lefgren's syndrome most often develops in young women (20-35 years old). Children, as a rule, do not get sick. Lefgren's syndrome begins acutely, and then gradually clinical manifestations may themselves go away. Often there is a favorable course of the disease.
In addition to the viral and bacterial theory of the development of sarcoidosis, there is also an opinion that the disease develops in people who have long been in contact with volatile chemicals in factories. Do not lose sight of the hereditary factor. That is why sarcoidosis is a polyetiological autoimmune disease.
Clinical manifestations
Lefgren's syndrome with sarcoidosis appears polysymptomatic. Clinical manifestations begin acutely. A person has erythema nodosum on the body, it has a tendency to spread to the limbs, back, stomach, neck. The nodes are oval, their diameter can reach up to 6 centimeters. It is worth noting that necrosis does not occur at the site of erythema formation.
The main manifestation is considered to be damage to the bronchial and tracheal lymph nodes. This is a great danger to the patient:
- cough appears;
- occasionally increases body temperature;
- feeling worse;
- night sweats occur;
- sleep quality worsens;
- weight loss occurs;
- the person is worried.
Later Lefgren's syndrome is characterized by joint damage. They become swollen painful, and mobility worsens. Objectively, it is possible to notice an increase in the lymph nodes of the neck, as well as in the armpits.
How to diagnose?
Lefgren's syndrome is established on the basis of laboratory diagnosis and examination of the patient by a general practitioner. Erythema nodosum is of great importance in the diagnosis of this form of sarcoidosis. However, the doctor must differentiate the disease from tuberculosis or dermatovenereological ailments.
A laboratory blood test will show:
- increase in ESR;
- leukocytosis;
- the appearance of gamma globulins.
On the x-ray picture, you can notice a characteristic enlarged lymph nodes, as well as fibrous deposits. This helps to ensure in case of lung damage.
A mild form requires bronchoscopy with an aim biopsy. When examining the lung tissue, it is possible to see the characteristic histological structure of sarcoidosis: elements of epithelioid granuloma are found.
The prognosis and complications of the disease
Lefgren's syndrome occurs individually. How long and what are the forecasts, it is possible to answer only according to certain criteria. It is known that the older the person, the worse the prognosis. If you do not go to the doctor on time, then the syndrome is delayed, and complications join in:
- bronchial obstruction;
- respiratory failure;
- development of a "pulmonary heart";
- emphysema;
- accession of bacterial flora.
Respiratory failure develops after bronchial obstruction. The patient's condition gradually worsens, as the lungs begin to be replaced by connective tissue, the process can stop spontaneously after 1-2 years.
Gradually, the heart begins to increase due to a lack of oxygen in the blood. However, one should not ignore the request for medical help, because now Lefgren's syndrome is treatable.
Treatment
Unfortunately, medics cannot completely cure sarcoidosis, since its etiology is not completely known. But doctors prescribe special therapy that can stop the development of the process, control Lefgren's syndrome. Treatment begins with the appointment of steroidal anti-inflammatory drugs to reduce immunity. Vitamin complexes, antioxidants, immunosuppressants are also prescribed.
The course of treatment lasts an average of 8 months. It all depends on the individual characteristics of the immune system. Mandatory is the follow- up of patients with a pulmonologist, ideally it lasts 5 years.
What is the prophylaxis?
In order to prevent the development of sarcoidosis, you should first monitor your lifestyle. Nutrition should be balanced, contain fats, proteins, carbohydrates in the optimal ratio.
People predisposed to sarcoidosis should give up smoking, contact with aggressive chemicals.