Sarcoidosis

November 25, 2011

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Sarcoidosis   is a multisystem disease, which has no established etiology. The disease is characterized by the formation noncaseating epithelioid cell granulomas . They appear in different tissues and organs, most often occur in lymph nodes   and lungs. To date, studies have shown that this disease does not strike the adrenal glands. Most often the disease is diagnosed in residents of the northern states of Western Europe. The bulk of the people who developed sarcoidosis - people aged 20 to 40 years, often women. This disease is most often seen in winter and spring. The first described the disease dermatologist England Hutchinson, this happened in 1869.

It is believed that at the origin of the disease is a combination of factors of various kinds - Environmental . Genetic . infectious , and immunological . Thus, granulomas occur in humans due to exposure parasites . mushrooms . products activities plant   and Allowed . compounds metals . Several viruses ( Borrelia burgdorferi . Mycobacterium tuberculosis . Chlamydia pneumoniae . Propionibacterium acnes . herpes virus   and adenoviruses ) Are considered the most pathogenic infectious agents.

The patient comes first and foremost sarcoidosis lymph nodes and lungs (in these bodies accounts for around 90% of sarcoidosis lesions), also suffers from the spleen, liver, and even more rarely lesions appear on the skin (subcutaneous nodules and erythema nodosum ) In front of ( keratoconjunctivitis . iridocyclitis ) For the joints and bones ( arthritis The appearance of a large number of cysts   bone phalanges), kidney ( nephrolithiasis . nephrocalcinosis ). Even more rarely, the disease can affect other organs. In this disease there is a chronic granulomatous inflammation type, which is characterized by delayed hypersensitivity. The most commonly occur in sarcoidosis granulomas epithelioid cell . macrophage . mononuclear . If during the pathological process develops fibrosis , The granuloma becomes acellular hyaline mass.

Symptoms of sarcoidosis

Defining the clinical picture of the disease, the expert primarily determines those bodies are currently involved in the pathological process, as well as the degree of impairment in their function and anatomy. Given that the most common manifestation of the disease is pulmonary sarcoidosis, it is important to make an immediate diagnosis of lung lesions.

In the process of diagnosing lung lesions by x-ray examination is customary to distinguish four stages:

• Stage 0   - There is a normal result of X-ray examination of the chest;
• Stage I   - An increase in regional lymph nodes, generally on both sides, often asymmetrically nodes increases;
• Step II   - There is a focal or miliary dissemination on both sides. It is associated with the roots of the lungs. Also, there may be infiltration mainly in the middle and lower parts of the lungs;
• Stage III   - During the study revealed widespread pulmonary fibrosis, which is characterized by large drain focal tumors.

As complications, typical for such phenomena as pulmonary sarcoidosis the second and third stages, characterized by the emergence obturation of the bronchi . pulmonary heart . emphysema . respiratory failure . There is also a conditional classification of sarcoidosis, according to which the share sharp . subacute   and chronic   the type of disease. When acute and subacute sarcoidosis may occur syndrome Lefgrena Which is accompanied by fever , Feeling of increased fatigue. It is also observed in patients with bilateral lymphadenopathy   polyarthralgia roots of the lungs, and erythema nodosum. Given symptoms of these types of sarcoidosis may be combined in different ways. Patients may complain of a wide range of manifestations of the disease. Quite rare so-called syndrome Heerfordt-Valdenstrema , Which is characterized by the manifestation fever . facial paralysis . This increases the parotid glands and anterior uveitis occurs. It is important to bear in mind that with the rapid progression of the disease occurs more rapidly and remission. In acute sarcoidosis in about 80% of cases there is spontaneous remission.

A different picture is observed in patients with chronic sarcoidosis. Where the disease is delayed, patients complain only a manifestation of weakness and higher shortness of breath .

Chronic sarcoidosis referred to as X-ray stages II, III. In this case, spontaneous remission occurs less frequently, about 30% of cases. If sarcoidosis progresses, the process can also be formed chronic pulmonary heart. Thus 10% of the cases death occurs.

Symptoms that significantly affect the quality of life of people who become ill with sarcoidosis, a constant feeling of fatigue. Accepted provide four different types of state of fatigue in this disease:

• fatigue, which manifests itself in the morning: the patient can not get out of bed;
• intermittent fatigue, which throughout the day forced to slow down the activity of the patient;
• fatigue evening at which a person has a lot of energy in the morning, but by evening he feels that force almost at the end;
• Chronic fatigue In which the patient manifests myalgia . weakness . depression , In the absence of physical pathology.

Very often, patients with sarcoidosis syndrome occurs, which has no specific explanation. This pain in the chest, with a different modality and lokalizinovannye in different places. In this manifestation of the relationship between the degree of pain and lymphadenopathy as well as changes in the thorax is not installed. Sometimes, during the inspection of patients diagnosed with the presence of erythema nodosum . As a rule, it is localized in the legs. Erythema painful on palpation, and in the waning of the skin retains the gray-purple color. In order to quickly establish the correct diagnosis, shown holding node biopsy, which increased. However, other invasive studies are no longer needed.

Diagnosis of sarcoidosis

To establish a diagnosis of "sarcoidosis", the method of exclusion. It is important to clearly differentiate the disease from Tuberculosis . carcinomatosis . Hodgkin . pneumoconiosis   and other lung diseases. Only the value of screening can have regular rentgenotomografiya executed without a series of longitudinal tomograms. Routine X-ray computer tomography also provides the necessary information for a clear diagnosis.

During the initial examination of the patient is necessary to carry out X-ray computed tomography or magnetic resonance. Wherein such methods should be investigated and the abdominal organs and the kidneys. Certain data can be obtained during the ultrasound examination. Such a survey can be carried out to the lymph nodes, and most other organs. To evaluate respiratory problems, it is important to carefully examine the respiratory function and determine the status of the diffusion capacity of the lungs of the patient.

It is also in the process of diagnosis of the disease is carried out a number of laboratory tests, during which determined the changes of indicators that may indicate the presence of inflammation in the body. Thus, it can be increased erythrocyte sedimentation rate . eosinophilia . leukocytosis . lympho - And monocytosis . giperalfa -, beta globulinemi I at the initial stage, followed by Hypergamma-globulinemiey High activity of some enzymes. Very often the patients in the presence of anemia. It is also in the process of laboratory tests can identify the signs of organ damage. Important for the diagnosis data can be obtained from histological studies of remote limfuzlah, particles of skin and bronchial mucosa, where there are elements of the granuloma.

Diagnosis is based on the presence of characteristic beskazeoznoy epithelioid cell granulomas, in a certain organ, as well as clinical and instrumental signs. This eliminates the presence of other diseases whose symptoms resemble sarcoidosis.

Complications of sarcoidosis

The most serious complication of the disease can become progressively developing respiratory insufficiency . And as a result of inflammation of the eye may develop blindness. Such complications are prevented by receiving corticosteroids .

Treatment of sarcoidosis

On average, 50-70% of cases of sarcoidosis identification process should be spontaneous remission. In view of the fact that after treatment with hormones significantly reduces the possibility of remission, after confirmation of the diagnosis "sarcoidosis" and provided that the clinical disease does not manifest itself, should not be carried out active treatment for 6-8 months from the time of detection of the disease. This requires constant monitoring of the patient. In the case of a slow regression of the disease or its lack of specialist assigns course glucocorticosteroids . Indications for such a course of therapy is the presence of clinical manifestations, disease progression, and combined lesions of the lymph nodes inside the chest (the lymph nodes greatly increase in size), generalized forms. Therapy is continued for 6-8 months. Thus except glucocorticosteroids administered to the patient with additional nonsteroidal anti-inflammatory action of drugs, and also immunosuppressants . cytostatics . antioxidants . TB   drugs. If the disease takes a serious form, it is used to treat plasmapheresis . radiation therapy . hemosorbtion . Also at the terminal stages of sarcoidosis have been successfully performed transplantation of the lungs, liver, heart and kidneys.

Prevention of sarcoidosis

To prevent flare-ups, you need to pay special attention to healthy lifestyle . If the patient is diagnosed with pulmonary sarcoidosis, then such a person is most important to stop smoking, which directly affects the difficulty in breathing and provokes complications of the disease. It is recommended not to take drugs and avoid contact with chemicals that are harmful to the liver, avoid toxic volatile substances, dust and gases that adversely affect the condition of the lungs. Patients with sarcoidosis always a high level of calcium in the blood that contributes to the appearance in the bladder and kidney stones. Given this, the patient should not eat foods high in calcium. To prevent relapses and complications of the disease, must be required to undergo regular courses of therapy and visit a specialist for regular monitoring of the health status.