pulmonary sarcoidosis - is a systemic disease, accompanied by the formation of granulomas, consisting of cell-Piragova Langhans and epithelial cells.The granulomas are also diagnostic feature that is detected by means of microscopic examination, but sarcoid nodules are not accompanied by caseous necrosis and tubercle bacilli are absent.Also, the nodules coalesce as they grow and form pockets of different sizes.
not only easy, but also affects many organs in sarcoidosis.Most often it is the lymph, intraperitoneal, tracheobronchial, bronchopulmonary nodes, spleen and liver.It is possible the defeat of bodies, bones, joints, nervous system, heart, parotid salivary glands of the skin.However, pulmonary sarcoidosis can occur long time without clinical manifestations.Also it is not transmitted from patient to patient and is not contagious.
etiology is unknown today.People of any age subject to disease, but pulmonary sarcoidosis in children is rare.We only know that pulmona
In sarcoidosis on the walls of the bronchial and pulmonary granulomas formed of two types:
• The first type - sclerosing or stamped.The granulomas of small size, with the border from the surrounding tissue, and connective tissue cells - fibroblasts surrounded by granulomas;
• The second type - large granulomas that do not have clear boundaries.
sarcoid granulomas Quite often confused with tuberculosis.To determine the exact diagnosis is necessary to conduct laboratory examination of tissue.
Depending on the location, the disease is divided into intrathoracic sarcoidosis glands and lungs, lymph nodes, respiratory system and other organ sarcoidosis generalized form.
Adrift disease is divided into:
- phase regression (reverse development, the process of remission).Accompanied by a regression of dispersal, and seal pretty rare calcification formed sarcoid granulomas in the lymph nodes and lung tissue;
- stabilization phase;
- acute phase, or active phase.
directly on the speed with which change is increasing, pulmonary sarcoidosis is divided:
- Chronic sarcoidosis;
- Slow sarcoidosis;
- Progressive sarcoidosis;
- abortive sarcoidosis.
reasons pulmonary sarcoidosis
Strange but true causes of pulmonary sarcoidosis are still unknown.Some scientists believe the disease is genetic, others that pulmonary sarcoidosis occurs due to disruption of the immune system.There is also the assumption that the cause of pulmonary sarcoidosis is a biochemical disturbances in the body.But at the moment most of the scholars of the opinion that the combination of these factors is the cause of sarcoidosis of the lungs, although none advanced the theory does not confirm the origin of the nature of the disease.
Scientists studying infectious diseases, suggest that the simplest, Histoplasma capsulatums, spirochetes, fungi, mycobacteria and other microorganisms are pathogens of pulmonary sarcoidosis.As well as endogenous and exogenous factors may be the cause of disease.Thus, today accepted that pulmonary sarcoidosis polietiologichesky genesis is associated with biochemical, morphological, immune disorder and genetic aspects.
observed incidence in people of certain professions: fire (because of increased exposure to toxic or infectious), mechanics, sailors, millers, agricultural workers, postal workers, workers of chemical production and health.It is also observed in the lungs of sarcoidosis patients with tobacco dependence.The presence of an allergic reaction to some substance perceived by the body as foreign because of a violation of immunoreactivity, does not exclude the development of pulmonary sarcoidosis.
cytokine cascade is the cause of granuloma.They may be formed in various organs, and also consist of a large number of T-lymphocytes.
A few decades ago, there was an assumption that pulmonary sarcoidosis is a form of tuberculosis, which is caused by weakened mycobacteria.However, according to recent data it found that they are different diseases.
Sarcoidosis of the lungs begins with the fact that in the pathological process involved the alveolar tissue and at the same time developing interstitial pneumonitis or alveolitis.
Symptoms of pulmonary sarcoidosis
pulmonary sarcoidosis have no clear clinical picture, as it is not uncommon asymptomatic.For example, the majority of patients hilar limfozhelezistaya form of the disease, clinically manifested.The most common pulmonary sarcoidosis is suspected in detecting lymphadenopathy roots of the lungs.Signs of pulmonary sarcoidosis include: nodular erythema, pain in the joints, fever, shortness of breath, cough, chest pain, restless sleep, insomnia, sweating at night.Also, there is often fever, weight loss, loss of appetite, fatigue, weakness, anxiety, severe malaise.
pulmonary sarcoidosis is divided into three stages: primary, mediastinal, pulmonary and pulmonary.
Symptoms of pulmonary sarcoidosis early stages are similar to the symptoms of many other diseases: unwarranted anxiety, fatigue, sleep disturbances, and so on. A frequent feature of pulmonary sarcoidosis is a fatigue that is felt in the morning (people it feels are not standing still in bed), and the secondafternoon.At this stage, as a rule, there is asymmetric and bilateral lymph node enlargement: tracheobronchial, paratracheal, bifurcation, bronchopulmonary.
second stage pulmonary sarcoidosis manifest symptoms characteristic of diseases of the respiratory tract: pain in the chest, joints, coughing, wheezing, shortness of breath, weakness.It is possible the development of inflammation in the subcutaneous fat of skin vessels.This stage is accompanied by bilateral pulmonary sarcoidosis dissemination (miliary, focal), infiltration of the lung tissue.
third stage includes a set of symptoms of the first and second stages of pulmonary sarcoidosis.However, there are enhanced wet and dry wheezing, pain in the affected area of lung, crispy and wheezing sounds, arthralgia.Also, the third stage is shown lymph node, parotid glands syndrome (Herford), eyes and other organs that are not associated with the respiratory system.It is possible the defeat of cranial nerves, the formation of cysts in the bones, liver enlargement.
last stage pulmonary sarcoidosis may manifest severe fibrosis or pneumosclerosis lung tissue, without an increase in intrathoracic lymph nodes.Increase of emphysema and fibrosis occurs in the formed discharge conglomerates during disease progression.Also, the disease manifested cardio-pulmonary insufficiency.
pulmonary sarcoidosis progression extrapulmonary symptoms manifested as affected surrounding tissue.
Sarcoidosis go beyond the lungs, affecting the spleen and liver, it is not clinically manifested.Ultrasound examination may show slight increase in internal organs.In the event 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 function of the spleen and liver are not violated.Occasionally develop cirrhosis and holeostaz.
differences between sarcoidosis and granulomatous hepatitis are not clear.Pretty rare gastric granulomas.Mesenteric lymphadenopathy causes pain in the abdomen.
Striking joints and bones, the disease does not manifest itself clinically, but in patients enzymes may be elevated.Sometimes it develops acute or mute myopathy accompanied by muscle weakness.Perhaps the onset of pain in motion.However, damage to the bones in sarcoidosis lung differ from arthritis that are less harmful to the joints and bones.It is possible the development of the roots of the lungs lymphadenopathy, erythema nodosum, acute arthritis, osteopenia.
If any damage to the myocardium, the main feature of the disease is episodic vertigo, will also be disturbed heart rhythm.It is possible the onset of sudden death in the case of strong compression of the heart muscle granulomas.Pulmonary arterial hypertension or cardiomyopathy contribute to the development of heart failure.Quite rarely develop pericarditis.
pulmonary sarcoidosis has a significant effect on the nervous system.There may be a loss of sensation, unilateral facial paralysis, swallowing more difficult, paralysis of limbs, dizziness.Neuropathy of the eighth cranial nerve leading to hearing loss.It is not excluded development optic neuropathy and peripheral neuropathy, polyphagia.
If pulmonary sarcoidosis underwent kidney damage, most often hypercalciuria.Also, developing nephrocalcinosis requiring a kidney transplant, nephrolithiasis caused by chronic renal failure and interstitial nephritis.
With the defeat of the visual organs, there is a feeling of burning, blush mucous membranes, increased sensitivity to light, watery eyes present.The disease is also accompanied by high blood pressure (intraocular).Developing secondary glaucoma, optic neuritis, dacryocystitis, chorioretinitis, iridocyclitis and conjunctivitis.In the absence of treatment progression leads to blindness, but usually resolves spontaneously.
When skin lesions on the body are formed reddish nodules of medium size.Quite rarely observed severe damage to the skin.Develops erythema nodosum: on the front surface of the lower extremity nodules appear solid red.For non-specific lesions include subcutaneous nodules, papules, macular spots, hyperpigmentation and hypopigmentation.It is possible the development of lupus perfrigeration: ears, lips, cheeks and nose appear protruding spots.
In sarcoidosis lymph nodes are not usually enlarged, only occasionally visible enlarged lymph nodes in the groin or neck.In some cases, common cervical or mild peripheral lymphadenopathy.
Stage pulmonary sarcoidosis
In its development of pulmonary sarcoidosis is divided into four stages:
• Stage 0 asymptomatic.Patients undergoing medical check-ups, the disease is not detected even on X-rays;
• the 1st stage lung tissue remains unchanged, but a small increase of intrathoracic lymph nodes;
• the 2nd stage of the pathological process is observed in the lung tissue, thoracic lymph nodes significantly increased;
• 3 stage is accompanied by significant changes in lung tissue, however, the lymph nodes are not increased;
• 4 stage accompanied by the formation of fibrosis - an irreversible process of densification of the lung tissue with the formation of scars on it (pulmonary connective tissue is replaced).
first three stages of clinically manifest.Patients can learn about the presence of pulmonary sarcoidosis only as a result of preventive radiological investigation during the inspection.The pictures will be noticeable changes in the lung tissue.Pretty rare, patients with early-stage pulmonary sarcoidosis, which the body temperature rises, limbs swollen joints, lymph nodes are enlarged.
diagnosis of sarcoidosis lung
pulmonary sarcoidosis is not so simple, however, it is possible, regardless of the stage.We need an accurate medical history of the patient, all of the clinical manifestations, laboratory blood tests (ESR acceleration, eosinophilia, leukocytosis, increased globulins).It is also necessary to carry out X-ray, ultrasound, computed tomography and magnetic resonance, bronchoscopy and biopsy with subsequent histological examination, radionuclide methods.On the necessity of ultrasound with fine needle biopsy of lymph nodes decides specialist.Always patient is assigned urinalysis and functional test of the kidneys and liver.Additional research will be scheduled in case of complications.
for acute pulmonary sarcoidosis flow characteristic changes in laboratory parameters of blood, which is indicative of the inflammatory process: a significant or moderate increase in erythrocyte sedimentation rate, lymph and monocytosis, eozofiliya.However, blood counts may be normal pulmonary sarcoidosis.Leukocytosis will occur, if struck by the bone marrow, spleen and liver.To exclude renal spend urine define functional tests (blood urea nitrogen, creatine).
more characteristic changes can be detected during X-ray examination.MRI and CT scan to determine the increase in tumor lymph nodes, especially in the root, dissemination focal fibrosis, emphysema, cirrhosis of the lung tissue.
In most patients, a positive response Kveim - after intradermal specific antigen (substrate sarcoid tissue of the patient) 0.2 ml, produced purple-red nodule.
During the biopsy with bronchoscopy can detect direct and indirect signs of pulmonary sarcoidosis: dilated blood vessels in the mouths of the equity of the bronchi, and sarcoid lesions of the mucous membranes (the presence of warty growths, bumps, patches), the symptoms of enlarged lymph nodes in the place of bifurcation, atrophic ordeforming bronchitis.
more reliable method of diagnosis of pulmonary sarcoidosis is a histological study of biological material taken during bronchoscopy, open lung biopsy, transthoracic puncture, preskalennoy biopsy, mediastinoscopy.The biological material experts define the elements of the granuloma (epithelioid) no signs of perifocal inflammation and necrosis.
angiotensin-converting enzyme (ACE) is a marker of activity in sarcoidosis and pulmonary its content in the blood is significantly increased.Also, elevated levels of calcium in the urine and the blood is an indication of the presence of complications in the body.
To eliminate TB, it is necessary to carry out the tuberculin Mantoux test.If the body's active form of pulmonary sarcoidosis, Mantoux test is usually negative, but there are exceptions.
Despite the fact that the diagnosis required a lot of medical procedures, it is the correct diagnosis allows to choose the right treatment.
Sarcoidosis of the lungs in the majority of patients is accompanied by spontaneous remission and for this reason, for 8 months the patient will be under surveillance.This allows you to determine the prognosis and the need for specific treatment.
As a rule, milder forms of the disease, which flow without deterioration, the treatment is not administered.Even in the case of minor changes in the lung tissue and satisfactory condition of the patient is carried out just watching.This is due to the fact that the granulomas which are formed in the lungs and pulmonary sarcoidosis absorbed passes independently.
Severe pulmonary sarcoidosis require no treatment, since there is a risk of complications, including death.It is possible the development of tuberculosis and serious diseases of other organs.
In case of pulmonary sarcoidosis is assigned a long course of anti-oxidants (Acetate, Tocopherol, Retinol and others), immunosuppressants (azathioprine, Rezohin, delagil), anti-inflammatory drugs (indomethacin), steroid agents (prednisolone).