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November 25, 2011
The sarcoidosis is a multisystem disease which has no the established etiology. Formation of epitelioidnokletochny nekazeifitsirovanny granulomas is characteristic of an illness. They appear in different fabrics and bodies, most often arise in lymph nodes and lungs. Today researches show that this illness does not affect adrenal glands. The most often this disease is diagnosed for residents of the northern states of Western Europe. The bulk of people at whom the sarcoidosis was shown is aged people from 20 to 40 years, women are more often. At the same time the disease is most often shown during the winter and spring period. The first a disease the dermatologist from England described Hutchinson, it happened in 1869.
It is considered to be that the combination of any factors — ecological, genetic, infectious, and also immunological is the cornerstone of developing of this illness. So, granulomas in a human body appear in view of influence of parasites, mushrooms, products of activity of plants and animals, compounds of metals. A number of viruses (Borrelia burgdorferi, Mycobacterium tuberculosis, Chlamydia pneumoniae, Propionibacterium acnes, a virus of herpes and adenoviruses) are considered as the most pathogenic infectious activators.
At the patient with a sarcoidosis first of all there comes defeat of lymph nodes and lungs (about 90% of defeats by a sarcoidosis are the share of these bodies), the spleen, a liver also suffers, even more seldom defeats are shown on skin (hypodermic small knots and a knotty erythema) in the eyes (a keratoconjunctivitis, an iridocyclitis), on joints and bones (arthritis, emergence of a large number of cysts in bones of phalanxes of fingers), kidneys (a nephrolithiasis, a nephrocalcinosis). Even less often the disease can affect other bodies. At this disease there is a granulematozny inflammation of chronic type of which the slowed-down hypersensitivity is characteristic. Most often at a sarcoidosis granulomas epithelioid and cellular, macrophagic, mononuclear are shown. If during pathological process fibrosis, then a granuloma develops to become acellular hyaline weight.
Defining a clinical picture of course of a disease, the specialist, first of all, defines those bodies which are involved in pathological processes now, and also extent of disturbances in their functions and anatomy. In view of the fact that the most frequent display of this disease is the sarcoidosis of lungs, it is very important to make immediate diagnosis of damages of lungs.
In the course of diagnosis of damage of lungs by x-ray inspection it is accepted to allocate four stages:
- the stage 0 – is observed a normal radiological consultation report of a thorax;
- the stage of I – is observed increase in regional lymph nodes, as a rule, from two parties, most often nodes increase asymmetrically;
- the stage of II – takes place miliary or focal dissimination from two parties. It is connected with roots of lungs. Also the infiltration generally in average and lower parts of lungs can be observed;
- the stage of III – during researches comes to light a widespread pneumosclerosis of which big drain focal new growths are characteristic.
As the complications characteristic of such phenomenon as a sarcoidosis of easy second and third stages, emergence of obturation of bronchial tubes, a pulmonary heart, emphysema of lungs is characteristic, respiratory insufficiency. There is also conditional classification of a sarcoidosis according to which divide acute, subacute and chronic type of an illness. At an acute and subacute sarcoidosis there can be a syndrome of Lefgren which is followed by fever, feeling of the increased fatigue. Also at patients the bilateral lymphadenopathy of roots of lungs a polyarthralgia and a knotty erythema is observed. The specified symptoms at these types of a sarcoidosis can be combined differently. Patients can complain of wider range of displays of an illness. Rather seldom the so-called syndrome of Heerfordta-Valdenstrem of which display of fever, paralysis of a facial nerve is characteristic meets. At the same time parotid lymph nodes increase and the front uveitis is observed. It is important to consider that at prompt progressing of an illness there comes also the remission stage more quickly. At an acute sarcoidosis approximately in 80% of cases spontaneous remission is observed.
Other picture is observed at patients with a chronic sarcoidosis. Here the course of a disease drags on, patients complain only of the increased weakness and display of short wind.
The chronic sarcoidosis is carried to x-ray stages of II, by III. In this case spontaneous remission arises much less often, approximately in 30% of cases. If the sarcoidosis progresses, then in process the chronic pulmonary heart can be also created. At the same time in 10% cases there is a lethal outcome.
Symptom which considerably influences quality of life of the people who got sick with a sarcoidosis is the constant feeling of fatigue. It is accepted to allocate four different types of a condition of fatigue at this disease:
- fatigue which is shown in the morning: the patient cannot be roused;
- the fatigue is intermittent which for day forces the patient to reduce rates of activity;
- the fatigue is evening at which at the person there is a lot of energy in the morning, but by the evening he feels that forces practically on an outcome;
- the fatigue is chronic at which at the patient the mialgiya, weakness, a depression, on condition of absence of physical pathology is shown.
Very often patients with a sarcoidosis have a syndrome which has no certain explanations. It is pain in a breast, with a different modality and lokalizinovanny in different places. At the same time dependence between manifestation of pain and degree of a lymphadenopathy, and also with the changes happening in a thorax is not established. Sometimes during survey existence of a knotty erythema is diagnosed for patients. As a rule, it is localized on shins. The erythema at a palpation is painful, and during process fading skin keeps gray-violet color. Quickly to establish the correct diagnosis, carrying out a biopsy of a node which increased is shown. At the same time other invasive researches will not be necessary any more.
Diagnosis of a sarcoidosis
To establish the diagnosis "sarcoidosis", the exception method is applied. It is important to differentiate accurately an illness with tuberculosis, a carcinomatosis, a lymphogranulomatosis, a pneumoconiosis and other diseases of lungs. The usual X-ray tomography executed without a series of longitudinal tomograms can have only screening value. Carrying out a usual x-ray computer tomography also does not give necessary information for accurate establishment of the diagnosis.
In the course of primary inspection of the patient it is necessary to carry out a x-ray computer or magnetic resonance tomography. At the same time by such methods it is necessary to investigate both abdominal organs, and kidneys. Certain data can be obtained also in the course of ultrasonic research. Such examination can be conducted both for lymph nodes, and for the majority of other bodies. To estimate respiratory disturbances, it is important to investigate carefully functions of external respiration and to define a condition of diffusion capacity of lungs of the patient.
Also in the course of diagnosis of an illness a number of laboratory researches during which all changes of indicators which can confirm existence in an organism of inflammatory processes are defined is conducted. So, it can be increase SOE, an eosinophilia, a leukocytosis, a lymph — and a monocytosis, hyper alpha, beta globulinemiya at an initial stage with the subsequent hypergammaglobulinemia, high activity of a number of enzymes. Very often at patients available anemia. Also in the course of laboratory researches it is possible to define signs of defeat of bodies. Data, important for statement of the diagnosis, can be learned from histologic researches of remote limfuzl, particles of skin and mucous membrane of bronchial tubes where there are granuloma elements.
The diagnosis is established on the basis of existence of a characteristic beskazeozny epithelioid and cellular granuloma in a certain body, and also kliniko-tool signs. At the same time existence of other diseases which symptoms remind a sarcoidosis is excluded.
Complications of a sarcoidosis
Gradually developing respiratory insufficiency can become the most serious complication of this illness. And owing to an inflammation of eyes the blindness can develop. Such complications are prevented by reception of corticosteroid hormones.
Treatment of a sarcoidosis
On average in 50-70% of cases identification of a sarcoidosis is followed by process of spontaneous remission. In view of the fact that after carrying out treatment by means of hormones the possibility of remission, after confirmation of the diagnosis "sarcoidosis" considerably decreases and provided that clinically the illness does not prove, it is necessary not to carry out active treatment for 6-8 months since detection of an illness. At the same time continuous supervision over the patient is required. In case of slow regression of an illness or its absence the specialist appoints a course of glucocorticosteroids. Indications to such course of therapy is existence of the expressed clinical manifestations, progress of an illness, the combined defeats of bodies and lymph nodes in a breast (at the same time lymph nodes strongly increase in sizes), generalized forms. Therapy continues for 6-8 months. At the same time except glucocorticosteroids to the patient appoint additional nonsteroid drugs with antiinflammatory influence, and also immunodepressants, cytostatics, antioxidants, antitubercular medicines. If the illness takes a severe form, then the plasma exchange, radiation therapy, hemosorption is applied to treatment. Also on end-stages of a sarcoidosis transplantation of lungs, a liver, heart, kidneys was already successfully made.
Prevention of a sarcoidosis
To avoid exacerbations of an illness, it is necessary to pay special attention to a healthy lifestyle. If the sarcoidosis of lungs is diagnosed for the patient, then it is the most important to such person to refuse smoking which directly influences difficulty of breath and provokes complications of an illness. It is recommended not to accept drugs and to avoid contact with the chemical means harmful to a liver, to avoid toxic volatiles, dust and gases which harmfully influence a condition of lungs. At patients with a sarcoidosis always the high level of calcium in blood that promotes emergence in a bladder and kidneys of stones. Considering it, patients should not eat products with the high content of calcium. Not to allow an aggravation and complication of an illness, it is regularly necessary to take required courses of therapy and to visit the specialist for regular control of a state of health.
Section: Diseases of a respiratory organs