Sarcoidosis - Causes, symptoms and treatment. MF.

Sarcoidosis is a disease in which many organs and systems can be affected, characterized by the formation in the affected organs of granulomas, which can be considered at a microscopic level. The most commonly affected lymph nodes, lungs and spleen. It is not infectious diseases and is not transmitted to others. Earlier, sarcoidosis was considered a rare disease, but with the modern improvement of diagnostic capabilities, a more recent look at this pathology of doctors, he ceased to be a rarity.

Causes of sarcoidosis

More common sarcoidosis is found in a younger population of 20 to 40 years. There is some predominance of women. This disease is known for more than 100 years, but its causes are still unknown. There are speculations that sarcoidosis causes various viruses, for example, such as herpesvirus. There is also the possibility that this disease is hereditary, or caused by various mycobacteriosis( therefore, sarcoidosis was previously performed by phthisiatricians, sarcoidosis was considered a type of tuberculosis).Perhaps, there are a number of factors that together lead to changes in the organism that lead to the development of clinical manifestations of sarcoidosis. At the present stage of the development of medicine, it can be said that sarcoidosis is an independent disease, with as yet undefined origin.

In accordance with the international classification of diseases, isolated:

1. Sarcoidosis of the lungs
2. Sarcoidosis of the lymph nodes
3. Sarcoidosis of the lungs with sarcoidosis of the lymph nodes
4. Sarcoidosis of other specified and combined localizations
5. Sarcoidosis, unspecified

Symptoms of sarcoidosis

be both acute and gradual, and asymptomatic. The asymptomatic course of the disease is more common in sarcoidosis of the intrathoracic lymph nodes , without affecting other organs. In such cases, the diagnosis of the disease occurs with a prophylactic fluorographic examination.

More often the disease occurs gradually with such common symptoms as lethargy, fatigue, which are typical for a variety of diseases. When involved in the process of the lungs, patients complain of shortness of breath, cough, back pain. After 2-3 weeks, these symptoms can completely disappear. With prolonged flow, these manifestations can persist for a long time. A dry cough with hard-to-recover sputum is characteristic. Later, with the development of fibrosis( compaction of lung tissue), respiratory and pulmonary-cardiac insufficiency develops.

There is an acute course of sarcoidosis - Lefgren's syndrome .It is rarely noted, in 10% of cases. It is characterized by an increase in temperature to 38-39 ° C, the appearance of erythema nodosum( inflammatory nodes that rise above the skin level, more often occurs in the legs, forearms, less often the face), swelling and joint pain, as well as bilateral hilar lymph nodes. These signs may manifest in incomplete volume and the disease will proceed in this case more erased.

Nodular erythema in sarcoidosis

In addition, there is also the Heerfordt Syndrome characterized by the defeat of the salivary glands and eyes.

Among extrapulmonary localizations of the process, is most often isolated from the peripheral lymph nodes, skin and subcutaneous tissue .Cervical and subclavian nodes, less often axillary and inguinal are enlarged, dense-elastic, mobile, the skin over them is not changed, not complicated by fistula. Lymphonoduses of the abdominal cavity can also be affected. In 5% of cases, the spleen and liver are enlarged by .In the blood increased bilirubin, aminotransferase activity. Skeletal lesion of the skeleton is rare, characterized by a typical radiographic pattern. Typically, the development of multiple foci of defusing bone tissue in the phalanges of fingers and feet, associated with the elution of calcium and is accompanied by an increased content of it in blood and urine tests. The defeat of the central nervous system is very rare, it is difficult.

In 20-30% of cases, the sarcoidosis of the heart occurs, which is asymptomatic. Defects can be detected with the help of ECG, ultrasound of the heart. The volume and size of the left parts of the heart decrease, the right ventricle, arrhythmias increase.

If the above symptoms occur, the patient must first turn to the general treatment network, where the therapist will prescribe the necessary tests and other methods of research. And if you suspect a sarcoidosis will send to a specialist - pulmonologist. Early detection and treatment of the disease provides a favorable prognosis and recovery. Otherwise, gross changes in pulmonary tissue, respiratory and pulmonary heart failure, blindness and other disorders leading to disability of the patient.

More common sarcoidosis with its symptoms is very similar to tuberculosis, but the reasons for the occurrence, and accordingly the treatment of these diseases are very different. And treatment with anti-TB drugs, and possibly self-treatment of the patient, can lead to deterioration of the state, and in special cases, death. Similarly, in its current sarcoidosis is similar to such diseases as: lymphogranulomatosis, central lung cancer, toxoplasmosis, brucellosis and a whole group of diseases, combined under the name "granulomatous".Therefore, timely access to a doctor, diagnosis and treatment by a specialist can prevent undesirable consequences.

Required tests for suspected sarcoidosis

The tests to be performed include: general blood test, blood biochemistry, Mantoux test, dyskintest, general sputum analysis, sputum examination on mycobacteria, fibroblochoscopy with biopsy, spirography, radiography( CT, MRI).

In the blood, anemia, leukopenia, lymphopenia, monocytosis, an increase in the rate of erythrocyte sedimentation( more often in acute course) can be detected. At the same time, in many patients, especially in sarcoidosis of the intrathoracic lymph nodes, changes in the blood are not significant, or absent. At a biochemical research it is possible to reveal an increase in fibrinogen, lipoproteins, C-reactive protein, in some patients there is disproteinemia. In 15% - 20% of patients there is an increase in the level of calcium in the blood and urine. Both these analyzes tell us about the degree of organ damage in sarcoidosis, the severity of the process.

The majority of patients with sarcoidosis have tuberculin anergy, which reflects the disorders of cellular immunity. Negative tuberculin test and dyskintest are more common in sarcoidosis with lung damage.

General sputum analysis and sputum analysis on mycobacteria will help us to conduct differential diagnosis with other lung diseases, such as: aspergillosis, tuberculosis. In rare cases, mycobacterium tuberculosis( 1% of the combined pathology of sarcoidosis and tuberculosis) is detected in the sputum analysis.

In fibrobronchoscopy of patients with sarcoidosis, various changes may appear. In connection with the compression of the bronchi with enlarged lymph nodes there is a narrowing of the lumen of the bronchi. Deformation of their walls, bulging of the bronchus wall into the lumen, "sarcoid ectasia" - expansion, tortuosity, thickening of the vessels in the form of a network or separate plexuses: spiders. Most patients have nonspecific endobronchitis, usually bilateral. Rarely, there are multiple tubercles. Plaques, granulation.

When biopsy in such areas with the help of cytological and histological methods can detect elements of sarcoid granuloma. In the morphological study of the bronchi, various granulomas are found: typical sarcoid and numerous lymphoid cell clusters. The most frequently used in our time: transbronchial intrapulmonary biopsy, mediastinoscopy, transbronchial biopsy of intrathoracic lymph nodes, videotoroscopy. In addition, a biopsy of the bronchi, peripheral lymph nodes, skin, liver, spleen and others involved in the process of the organs is performed. The elements of sarcoidosis that are found in the biopsy material allow correctly and timely diagnosis.

Examination of the functions of external respiration( spirography, spirometry) help to identify violations of varying degrees in patients with sarcoidosis of the respiratory system. The degree of violation of bronchial patency, an increase in bronchial resistance to air flow, a change in the elastic properties of lung tissue suggests a progression of respiratory failure. Violations of obstructive nature are more frequent in patients with acute disease, restrictive, in chronic course.

One of the main methods of diagnosing sarcoidosis of intrathoracic lymph nodes and lungs is radiography. Lymph nodes are enlarged from both sides almost always. As a rule, bronchopulmonary lymph nodes are more affected. Shadows of the lymph nodes look like large conglomerates on the roentgenogram, but they can also be in the form of separate groups of lymph nodes. The greatest changes in the combined lesions of intrathoracic lymph nodes and lungs in the acute phase occur in the middle and lower sections of the lungs, and foci of dissemination are noted. They can be of different sizes. In some cases, the right changes are more pronounced than on the left. In the chronic course of the process, there are: strengthening of the pulmonary pattern, emphysema of the pulmonary fields, education of the "bull" type. Against this background, fresh foci may also appear.

Sarcoidosis of the intrathoracic lymph nodes on the x-ray

Isolated lung involvement is not observed often. After the treatment of sarcoidosis on the radiograph, residual changes in the form of pneumofibrosis of different degree of severity, cavity formations of the bullous type can be revealed.

The classification of stages of sarcoidosis associated with radiographic signs is used, it is distinguished by: 1 stage - an increase in the intrathoracic lymph nodes, 2 stage - development of combined lesions of the intrathoracic lymph nodes and lungs. Stage 3 - combined lesion of intrathoracic lymph nodes and lungs with the development of fibrosis and large formations.

All set of diagnostic methods, anamnesis of the disease, anamnesis of the patient's life leads to an accurate diagnosis.

Treatment of sarcoidosis

In the treatment of sarcoidosis, corticosteroids, non-steroidal anti-inflammatory drugs, pentoxifylline, cytostatics( methotrexate, azathioprine), immunosuppressants( cyclosporine), immunomodulating agents, physiotherapy methods, plasmapheresis are used. Currently, a number of hormone therapy schemes are used that reduce inflammation, improve the clinical picture of the disease. As a rule, prednisolone is prescribed, or its analagi( metipred).Inhalation corticosteroids, budesonide, are also used. Bekotid, beclomethasone dipropionate. They selectively affect the affected mucosa of the bronchi.

The complex treatment regimen for sarcoidosis includes delagil or plakvenil, they reduce the need for tissues in oxygen, inhibit the development of granulation tissue, are immunomodulators. During the entire treatment period, antioxidants are prescribed: vitamin E, sodium thiosulfate, vitamin C.

Patients with acute onset of the disease and microcirculation disorder are prescribed angioprotectants. It is necessary to avoid stress, avoid prolonged exposure to sun and hypothermia. In most patients with mild symptoms of the disease, with a moderate degree of increase in the intrathoracic lymph nodes, in the absence of pulmonary lesions, observation is usually prescribed for 3-6 months with the appointment of non-steroidal anti-inflammatory drugs and antioxidants: indomethacin, vitamin E, sodium thiosulphate, pentoxifylline, antimalarials( chloroquine).More often after such treatment, patients have full or almost complete remission. Rarely there is a worsening of the process against the background of treatment, which forces us to prescribe hormonal treatment. In sarcoidosis with limited damage to other organs, treatment with an antimalarial drug with minimal doses of the hormone is prescribed. In generalized forms, methotrexate, azathioprine or cyclosporine are also used.

Self-medication is not recommended. The whole period of rehabilitation must be strictly controlled under medical supervision. X-ray monitoring is conducted every two months.

At the first stage of treatment, especially in the acute stage of the disease, inpatient treatment is recommended for the selection of an individual treatment regimen, the selection of doses of hormone therapy. Treatment will last about two months, with the transition to outpatient monitoring. With not systematized treatment, the patient's condition can worsen, progress. The timing of treatment depends on the improvement of the patient's condition, analyzes, radiographic methods of investigation and depends on the degree of the process. Treatment for neglected cases can last up to two years.

Nutrition and lifestyle in sarcoidosis

The diet for sarcoidosis is not specific, but there are a number of limitations. Since the process is inflammatory in nature, and carbohydrates are beneficial for inflammation, it is necessary to exclude from their diet: sugar, sweets, cakes, products from yeast dough, sweet fizzy drinks. Any inflammatory process is also enhanced by the use of: sharp, salty and fried foods. It is necessary to exclude products containing calcium, as its content increases in blood and urine( milk and sour milk products, cottage cheese, sour cream).The food should be high-grade and easily assimilable: on pair, prepared by a method of cooking or suppression. It is useful to eat the fruits of sea-buckthorn, black currant, gooseberries, cherries, pomegranates, onions, garlic, sea cabbage, buckwheat and oatmeal, beans, peas. With hormone therapy should be limited intake of salt, as there is a fluid retention in the body. The protein breaks down, so you need to use a protein diet.

Folk methods of treatment

Folk methods of treatment include herbal treatment( root of althea, calendula, plantain, oregano, sage), if they are not allergic. In principle, such treatment is allowed, it does not lead to a violation of organs and systems. But, for example, treatment of vodka with oil, can affect the function of the liver, which in sarcoidosis may worsen. Badger fat contributes to the deterioration of the process in the lungs, the intensification of the inflammatory process, its use in sarcoidosis will only worsen the situation. Aloe, honey are natural immunomodulators, so in the complex treatment of sarcoidosis they can be used.

Rehabilitation after treatment of sarcoidosis

Non-drug rehabilitation: interstitial electrophoresis, magnetotherapy, heparin or lidase electrophoresis, ultrasound, laser therapy, physiotherapy, manual therapy. The duration of the overall treatment depends on the course of the disease and the condition of the patient.

Complications of sarcoidosis

The most common complications of sarcoidosis are: severe emphysema, respiratory failure, pulmonary heart disease, broncho-obstructive syndrome, pulmonary heart. That is, the emergence of irreversible processes, which subsequently become chronic and accompany the patient for life, require treatment. Therefore, timely treatment to the doctor, the annual passage of the fluorographic survey contributes to the early detection of the disease and excludes the appearance of complications.

Prognosis for sarcoidosis

Approximately 60% of patients develop spontaneous remission after two years.25% of patients completely recover after treatment. In 10% of cases, remission is not achieved. Treatment of extrapulmonary sarcoidosis and the central nervous system is often ineffective. Sarcoidosis is rarely the cause of a persistent loss of disability, especially with the current level of medicine. More often disability is associated with the development of pulmonary heart disease, pulmonary heart, as a consequence of a long-term sarcoidosis process. The lethal outcome occurs rarely - in 0.5-7% of cases.

Prevention of sarcoidosis

Since the possible causes of sarcoidosis are not fully known, it is difficult to talk about the prevention of this disease. You can talk about the observance of a healthy lifestyle: to exclude smoking and alcohol, prolonged exposure to the sun, avoid contact with chemicals, vapors, substances that affect the function of the liver and lungs. Pass an annual fluorographic study.

Doctor phthisiatrist Kuleshova LA