Sarcoidosis of the lungs is a systemic disease accompanied by the formation of granulomas, consisting of Piragov-Langhans cells and epithelial cells. Granulomas are also a diagnostic sign, which is detected by microscopic examination, however, sarcoid nodules are not accompanied by caseous necrosis and tuberculous mycobacteria are absent. Nodules also merge as they grow and form pockets of different sizes.
Not only the lungs, but also many organs are affected in sarcoidosis. Most often it is lymphatic, intrathoracic, tracheobronchial, bronchopulmonary nodes, spleen and liver. It is possible that the organs of vision, bones, joints, nervous system, heart, parotid glands, skin are damaged. However, sarcoidosis of the lung can take a long time without clinical manifestations. Also, it is not transmitted from the patient to the patient and is not contagious.
Etiology is unknown for today. People of any age are susceptible to the disease, however, sarcoidosis of the lungs in children is rare. It is only known that sarcoidosis of the lungs has racial and geographic features. For example, for 100,000 blacks, 36-64 people who have sarcoidosis in the United States have 100-14 light-skinned populations 10-14 cases. In Europe, there are 40 cases per 100 000 people, however, the incidence in the Nordic countries is much higher.
With sarcoidosis on the bronchial walls and in the lungs, granulomas of two types are formed:
• The first type is sclerosed or stamped. Granulomas of small size, which have a border from surrounding tissues, as well as cells of connective tissue - fibroblasts surround the granulomas;
• The second type is large granulomas that do not have clear boundaries.
Quite often, sarcoid granulomas are confused with tuberculosis. To accurately determine the diagnosis it is necessary to conduct a laboratory study of tissue.
Depending on the location, the disease is divided into sarcoidosis of the intrathoracic glands and lungs, lymph nodes, respiratory system with lesions of other organs and sarcoidosis of the generalized form.
On the course of the disease is divided into:
- The phase of regression (reverse development, process silencing). Accompanying the reverse development of resorption, densification and rarely enough calcification of sarcoid granulomas formed in lymph nodes and lung tissue;
- Stabilization phase;
- Aggravation phase or active phase.
Directly on the speed with which changes are increasing, sarcoidosis of the lungs is divided:
- Chronic sarcoidosis;
- Delayed sarcoidosis;
- Progressive sarcoidosis;
- Abortive sarcoidosis.
Causes of sarcoidosis of the lungs
Strangely enough, the true causes of sarcoidosis of the lungs are still unknown. Some scientists consider the disease genetic, others that sarcoidosis of the lungs arises from the impaired work of the human immune system. There are also suggestions that the cause of the development of sarcoidosis of the lungs is a biochemical disorder in the body. But at the moment, most scientists hold the view that the combination of the above factors is the cause of the development of lung sarcoidosis, although none of the advanced theory confirms the nature of the origin of the disease.
Scientists studying infectious diseases assume that protozoa, histoplasm, spirochetes, fungi, mycobacteria and other microorganisms are the causative agents of lung sarcoidosis. And also endogenous and exogenous factors can be the cause of the development of the disease. Thus, today it is considered that sarcoidosis of light polyethic origin is associated with biochemical, morphological, immune disturbance and genetic aspect.
There is a morbidity rate in people of certain specialties: firefighters (due to increased toxic or infectious effects), mechanics, sailors, millers, agricultural workers, postal employees, chemical workers and health care workers. Also, sarcoidosis of the lungs is observed in people with tobacco dependence. The presence of an allergic reaction to certain substances that are perceived by the body as foreign due to impaired immunoreactivity does not exclude the development of sarcoidosis of the lungs.
Cascade of cytokines is the cause of the formation of sarcoid granuloma. They can form in various organs, and also consist of a large number of T-lymphocytes.
Several decades ago, there was a suggestion that sarcoidosis of the lungs is one of the forms of tuberculosis, which is caused by weakened mycobacteria. However, according to recent data, it is established that these are different diseases.
Sarcoidosis of the lungs begins with the involvement of the alveolar tissue in the pathological process and the development of interstitial pneumonitis or alveolitis.
Symptoms of sarcoidosis of the lungs
In sarcoidosis of the lungs there is no clear clinical picture, since often there is its asymptomatic course. For example, in most patients, the intrathoracic lymphoid-leukemic form of the disease is not clinically apparent. Most of the sarcoidosis of the lung is suspected in the presence of lymphadenopathy of the roots of the lungs. Signs of sarcoidosis of the lungs are as follows: nodal erythema , joint pain, fever, shortness of breath, cough with phlegm, chest pain, restless sleep, insomnia, sweating at night. Also often there is fever, weight loss, loss of appetite, increased fatigue, weakness, anxiety, severe malaise.
Sarcoidosis of the lung is divided into three stages: primary, mediastinal pulmonary and pulmonary.
Symptoms of early stage sarcoidosis are similar to those of many other diseases: unreasonable anxiety, weakness, sleep disturbances, etc. A frequent sign of sarcoidosis of the lungs is fatigue, which is felt in the morning (a person feels it without getting out of bed), and in the afternoon . At this stage, as a rule, there is an asymmetric and bilateral increase in lymph nodes: tracheobronchial, paratracheal, bifurcation, bronchopulmonary.
The second stage of sarcoidosis of the lungs is manifested by symptoms characteristic of respiratory tract diseases: pain in the chest, in the joints, coughing, wheezing, shortness of breath, weakness. It is not excluded the development of the inflammatory process in the subcutaneous fat of the cutaneous vessels. This stage of sarcoidosis of the lungs is accompanied by bilateral dissemination (miliary, focal), infiltration of lung tissue.
The third stage includes a combination of symptoms of the first and second stages of sarcoidosis of the lungs. However, there are intensified wet and dry rales, pain in the affected area of the lungs, crunchy and wheezing sounds, arthralgia. Also, the third stage is manifested by the defeat of lymph nodes, parotid glands (Herford's syndrome), eyes and other organs that are not associated with the respiratory system. It is not excluded the defeat of the brain nerves, the formation of cysts in the bones, the enlargement of the liver.
The last stage of sarcoidosis of the lungs can be manifested by severe fibrosis or pneumosclerosis of the lung tissue, and there is no increase in the intrathoracic lymph nodes. The growth of emphysema and pneumosclerosis is due to the formed conglomerates, during the progression of the disease. Also, the disease manifests itself in cardiopulmonary insufficiency.
Sarcoidosis of the lungs as it progresses manifests itself as extrapulmonary symptoms, as adjacent tissues are affected.
Sarcoidosis leaving the lungs, hitting the spleen and liver, is not clinically apparent. Ultrasound examination can show a slight increase in internal organs. In the case of a significant increase in the liver, the patient feels heaviness in the right upper quadrant. The patient will complain of loss of appetite, but the functions of the spleen and liver will not be disturbed. Occasionally, cirrhosis of the liver and cholestasis develop.
Differences between granulomatous and sarcoid hepatitis are unclear. Quite rare are gastric granulomas. Mesenteric lymphadenopathy causes pain in the abdomen.
Affecting the joints and bones, the disease is not clinically apparent, but in patients the enzymes can be increased. Sometimes acute or mute myopathy develops, accompanied by muscle weakness. Perhaps the appearance of pain when moving. However, bone lesions in sarcoidosis of the lungs differ from arthritis in that they less harm the joints and bones. It is not excluded the development of lymphadenopathy of the roots of the lungs, nodal erythema, acute polyarthritis, osteopenia.
If there are damage to the myocardium, the main symptom of the disease will be episodic dizziness , and the heart rhythm will also be disturbed. It is not excluded the sudden death in the case of a strong compaction of granulomas of the heart muscle. Pulmonary arterial hypertension or cardiomyopathy contributes to the development of heart failure. Pericarditis rarely develops.
Sarcoidosis of the lungs has a significant effect on the nervous system. There may be loss of sensitivity, unilateral facial paralysis, swallowing is more difficult, paralysis of the limbs, dizziness. Neuropathy of the eighth cranial nerve leads to hearing loss. It is not excluded the development of neuropathy of the optic nerve and peripheral neuropathy, polyphagia.
If, in sarcoidosis of the lungs, the kidneys were damaged, hypercalciuria most often occurs. Nephrocalcinosis, requiring renal transplantation, nephrolithiasis caused by chronic renal failure and interstitial nephritis also develops.
When the visual organs are damaged, a burning sensation arises, the mucous membranes become red, sensitivity to light is increased, and lacrimation is present. The disease is also accompanied by increased pressure (intraocular). Secondary glaucoma, optic neuritis, dacryocystitis, chorioretinitis, iridocyclitis and conjunctivitis develop. In the absence of treatment, progression leads to blindness, but is most often resolved spontaneously.
With skin lesions, reddish nodules of medium size are formed on the body. Severe damage to the skin is rarely observed. Nodular erythema develops: on the front surface of the lower limb, solid nodules of red color appear. Nonspecific lesions include subcutaneous nodules, papules, maculae, spots, hyperpigmentation, and hypopigmentation. It is not excluded the development of cholerotic lupus: on the ears, lips, cheeks and nose appear protruding spots.
In sarcoidosis, lymph nodes are usually not enlarged, only enlarged lymph nodes in the groin or on the neck are occasionally seen. In some cases, there is cervical or mild peripheral lymphadenopathy.
Stages of sarcoidosis of the lungs
In its development, sarcoidosis of the lung is divided into four stages:
• 0 stage is asymptomatic. Patients undergoing preventive medical examinations will not be diagnosed even on X-rays;
• at the 1 st stage lung tissue remains unchanged, but there are small increases in the intrathoracic lymph nodes;
• at the 2 nd stage, a pathological process is observed in the lung tissue, the hilar lymph nodes are significantly enlarged;
• Stage 3 is accompanied by significant changes in lung tissue, however, lymph nodes do not increase;
• Stage 4 is accompanied by the formation of fibrosis - an irreversible process of tightening the lung tissue with the formation of scars on it (lung tissue is replaced by a connective).
The first three stages are not clinically apparent. Patients can learn about the presence of sarcoidosis of the lungs only on the basis of the results of a prophylactic X-ray examination during the examination. Changes in lung tissue will be noticeable in the pictures. Rarely are there patients with early stages of sarcoidosis of the lungs, in which the body temperature rises, the limb joints swell, the lymph nodes are enlarged.
Diagnosis of pulmonary sarcoidosis
Diagnosing sarcoidosis of the lungs is not so simple, however, it is possible, regardless of the stage. It requires an accurate medical history, all clinical manifestations, laboratory blood tests (acceleration of ESR, eosinophilia, leukocytosis, increase in globulins). It is also necessary to perform X-ray, ultrasound, computer and magnetic resonance tomography, biopsy with bronchoscopy and with further histological examination, radionuclide methods. The specialist decides on the need for an ultrasound study with fine needle biopsy of the lymph nodes. Always the patient is assigned a general urine test and a functional test of the kidneys and liver. An additional study will be scheduled in case of complications.
The acute course of sarcoidosis of the lungs is characterized by a change in the laboratory blood index, which indicates an inflammatory process: a significant or moderate increase in ESR, lympho- and monocytosis, and eosophilia. However, blood counts may be normal in sarcoidosis of the lungs. Leukocytosis will be manifested if the bone marrow, spleen and liver are affected. To exclude renal damage, urine tests are performed, functional tests are determined (blood urea nitrogen, creatine).
More characteristic changes can be detected during X-ray examination. MRI and CT of the lungs can detect tumor-like enlargement of the lymph nodes, especially in the root, focal disseminations: fibrosis, emphysema, cirrhosis of lung tissue.
Most patients have a positive Kveim reaction - after intradermal injection of a specific antigen (substrate of the patient's sarcoid tissue) 0.2 ml, a crimson-red nodule is formed.
During the biopsy with bronchoscopy, direct and indirect signs of sarcoidosis of the lungs can be detected: dilated vessels in the lobes of the lobar bronchi, as well as sarcoid lesions of their mucous membranes (the presence of warty growths, tubercles, plaques), signs of enlarged lymph nodes in the bifurcation site, atrophic or deforming bronchitis .
A more reliable method of diagnosing sarcoidosis of the lungs is histological examination of biological material taken during bronchoscopy, open lung biopsy, transthoracic puncture, pre-stained biopsy, mediastinoscopy. In the biological material, specialists determine the elements of the granuloma (epithelioid) without signs of perifocal inflammation and necrosis.
Angiotensin-converting enzyme (ACE) is a marker of activity of the process and in sarcoidosis of the lung its content in the blood is significantly increased. Also, an elevated level of calcium in the urine and blood is evidence of the presence of complications in the body.
In order to exclude tuberculosis , it is necessary to conduct a tuberculin test of Mantoux. If the body has an active form of lung sarcoidosis, the Mantoux test is usually negative, however, there are exceptions.
Despite the fact that for the diagnosis is required to conduct a lot of medical manipulations, it is the correct diagnosis that allows you to choose the right treatment.
Treatment of pulmonary sarcoidosis
Sarcoidosis of the lungs in most patients is accompanied by spontaneous remission and for this reason, the patient will be monitored for 8 months. This allows you to determine the prognosis and the need for specific treatment.
As a rule, for mild forms of the disease, which proceed without deterioration, treatment is not prescribed. Even in the case of minor changes in pulmonary tissue and a satisfactory condition of the patient, only observation is carried out. This is due to the fact that the granulomas that formed in the lungs dissolve and the sarcoidosis of the lungs passes by itself.
Severe forms of pulmonary sarcoidosis require treatment, since there is a risk of complications up to a lethal outcome. The development of tuberculosis and serious diseases of other organs is not ruled out.
In the case of lung sarcoidosis, a long course of antioxidants (Acetate, Tocopherol, Retinol and others), immunosuppressants (Azathioprine, Rezokhin, Delagil), anti-inflammatory drugs (Indomethacin), steroid agents (Prednisolone) is prescribed. If the patient is intolerant of Prednisolone, then non-steroidal anti-inflammatory drugs (Nimesulide, Diclofenac) are prescribed. On average, the course of treatment lasts 8 months, however, in case of severe disease, this period may be longer. In rare cases, specialists prescribe anti-tuberculosis drugs.
As a rule, during the first 4 months, prednisolone should be taken at 30-40 mg per day, after which the dosage is reduced to 5-10 mg. Take this drug is necessary for several months. After 24-48 hours the doctor prescribes glucocorticosteroid preparations in case of side effects on Prednisolone. Also in the course of treatment include anabolic steroids and potassium preparations (Nerobol, Retabolil).
Treatment always depends on the activity, progression and severity of the course of sarcoidosis of the lungs. In the case of combination therapy, which includes Dexamethasone or Prednisolone, the drugs alternate with non-steroidal anti-inflammatory drugs (Indomethacin, Voltaren).
In rare cases, inhaled glucocorticoids are prescribed for severe coughing. They contribute to the reduction of cough in patients with endobronchial lesions. In rare cases, lesions of the eyes and skin will be assigned to local glucocorticoids.
Clinical follow-up of patients is conducted by a phthisiatrician. Patients with pulmonary sarcoidosis are divided into two dispensary groups:
♦ The first group includes patients with active disease;
The IA group includes people whose disease is first diagnosed;
The IB group includes people whose condition has worsened, relapses after a prescribed course of treatment;
♦ The second group includes people who have an inactive form of the disease.
Patients also need to pay special attention to diet. Table salt should be limited and eaten as much as possible products enriched with proteins. In order to restore immunity to therapy, it is necessary to include medicinal and edible plants that concentrate certain BAS (biologically active substances) - zinc, manganese, silica and other minerals.
It is necessary to eat food plants that have immunocorrective properties - chokeberry, raw sunflower seeds, a decoction of young shoots of buckthorn sea-buckthorn, walnuts, sea cabbage, noble laurel, pomegranates, basil, legumes, leaves and black currant fruit. From the daily diet should be excluded the following products: dairy products, cheese, sugar, flour.
Sarcoidosis of the lungs in children is also treated by a phthisiatrist. The medication course is selected individually, depending on the condition of the child. With a view to prevention, it is necessary to temper the child, to accustom him to daily physical education, to monitor his communication to prevent pulmonary diseases. It is also necessary to include vegetables and fruits in his daily diet. Children who have had sarcoidosis of the lungs need to be explained that in the future they should not start smoking. Parents should protect the child from various contacts with chemicals. Many cleaning products contain a large number of chemicals that a child should not breathe.
Also, many patients in the treatment course include folk remedies. For example, from medicinal herbs (calendula, goraltea, sage, oregano), a decoction is prepared at home and taken 3 times a day for 1.5 months before meals, 50 ml each. Also popular is tincture of vodka and vegetable oil. It is mixed by 50 ml and taken 3 times a day throughout the year. Cases of complete recovery due to this tincture were noted. Still it is possible to dilute in a warm water of 20% a tincture of a propolis and on a glass of water of 10-15 gramme of the agent will be enough. Take it within 15 days 40 minutes before eating.
Most patients in the early stages of the disease prefer treatment with folk remedies. In the case of disease progression, such methods become ineffective. Every patient should understand that most herbs have a side effect. It is for this reason that the treatment of sarcoidosis of the lungs with folk remedies is usually the cause of the deterioration of the general condition.
Since sarcoidosis of the lungs is rarely diagnosed, a special diet has not yet been developed, however, a healthy lifestyle should be maintained. Sleep and nutrition should be full. It is recommended to stay out in the open air for as long as possible and exercise. However, direct contact with sunlight should be avoided (sunbathing is strictly prohibited). Also avoid contact with vapors of chemical liquids, dust, gases.
Prognosis of pulmonary sarcoidosis
As a rule, the symptoms of sarcoidosis of the lungs pass without treatment. In 60% of cases after 9 years, patients are not detected after diagnosis. After a few months, extensive inflammation of the lungs and enlargement of the lymph nodes may disappear. About 75% of patients who have only lymph node enlargement and only lung damage, recover completely within 5 years.
The most beneficial effects of pulmonary sarcoidosis are for patients in whom the disease has not spread beyond the chest, especially if it began with erythema nodosum. In 50% of cases there are relapses.
Despite the fact that quite often patients recover spontaneously, the manifestations and severity of lung sarcoidosis are quite variable. In most cases, a repeated course of glucocorticoids is necessary. It is for this reason that regular monitoring is necessary to detect relapses. In 90% of cases when spontaneous recovery occurs, relapses develop in the first two years after diagnosis. In 10% of cases, relapses are found after two years. Patients with no remission within two years will have a chronic form of lung sarcoidosis.
Sarcoidosis of the lungs is usually considered chronic in 30% of patients and 10-20% of its constant course. The disease is considered fatal in 5% of cases. The most common cause of death is pulmonary fibrosis with respiratory failure, after which pulmonary hemorrhage follows due to aspergilloma.
More unpleasant consequences of sarcoidosis of the lungs occur in patients with extrapulmonary disease and in individuals of African-American race. In 89% of cases, recovery occurs in European countries. Signs of a favorable outcome is the presence of acute arthritis and nodal erythema. However, adverse signs of pulmonary sarcoidosis are: extensive pulmonary disease, myocardial diseases, nephrocalcinosis, neurosarcoidosis, chronic chronic hypercalcemia, uveitis. In 10% of cases, respiratory and eye injuries develop.