Sarcoidosis

Description:

Sarcoidosis (disease of Benye-Beka-Shaumana) - a general disease of not clear etiology which pathomorphologic basis is made by epithelioid and cellular granulomatozny structure without signs of a caseous necrosis.

Sarcoidosis symptoms:

The sarcoidosis is shown preferential at advanced age; more often women are ill.

At a sarcoidosis visceral bodies are surprised: lungs, bones, joints, organs of sight, endocrine glands, lymph nodes, nervous system. As the most frequent specific manifestations of a sarcoidosis serve the mediastinitis and miliary damages of lungs (to 95% of patients). At roentgenoscopy increase and consolidation of intrathoracic lymph nodes, the increased netting of the pulmonary drawing and multiple focal blackouts are defined. Damages of bones - a multiple pseudocystic osteitis (ostitis multiplex cystoides of N. V. Morozov-Yunglinga) which is shown a spindle-shaped thickening of phalanxes and spontaneous fractures are characteristic. Radiological find the roundish centers of an osteoporosis.
Eyes are quite often affected. The iritis, an iridocyclitis, a chorioretinitis develop. The phenomena of an iridocyclitis are sometimes combined with parotitis, a dacryocystitis (Heerfordt's syndrome).

Find increase in a liver, spleen, parotid, submaxillary and peripheral lymph nodes, damage of kidneys, appendages of testicles in many patients.

In blood note a hyperproteinemia, a hyperglobulinemia, a hypercalcemia, increase in level of alkaline phosphates, increase in SOE (at the disseminated sarcoidosis). Body temperature usually does not increase.

The stage of I is characterized by increase in intrathoracic lymph nodes. At the II stage along with it the expressed intersticial changes and the centers of various size preferential in average and lower parts of lungs are defined. In the III stage come to light considerable diffusion fibrosis in lungs and large, usually drain focuses, and also expressed. emphysema quite often with violent and dystrophic and bronkhoektatichesky cavities and pleural consolidations. Approximately one third sick at I and II stages has no noticeable subjective frustration and the disease comes to light at a X-ray fluorographic research. Current subacute or chronic, quite often wavy; weakness, subfebrile condition, a stethalgia, dry cough, a loss of appetite are noted. Rattles in lungs are listened seldom and in a small amount. Sometimes the sarcoidosis begins sharply with high temperature, swelling of joints of extremities, emergence of a knotty erythema, mainly on skin of shins, increases in outside lymph nodes.

Sarcoidosis reasons:

The views of sarcoids dominating earlier as display of a tuberculosis infection were shaken by V. H. Vasilenko's researches and another indicated on:

  1. the fundamental difference of a sarkoidny granuloma from a tubercular hillock consisting in the expressed proliferative character and an outcome in a hyalinosis;
  2. rarity of reliable detection of tubercular mycobacteria;
  3. negative takes of tuberkulinovy tests.

Authors came to a conclusion that the sarcoidosis is the independent disease relating to diseases of reticuloendothelial system.

Treatment of the Sarcoidosis:

Corticosteroids remain a treatment basis. Lyofgren's syndrome usually does not demand treatment, except for cases when non-steroidal anti-inflammatory drugs do not stop symptomatology. In such cases the short course of treatment Prednisolonum in a dose of 20 mg/days can be effective before disappearance of symptoms. It is not reached consensus on when to begin use of glucocorticosteroids who has to receive them as long and in what doses. Usually begin treatment with Prednisolonum for control of symptoms, at treatment of damages of heart, kidneys and a nervous system. Finds support and opinion that hormones should be used if within 3-6 months changes on roentgenograms of bodies of a thorax do not disappear. Inhalations of high doses of a budesonid or flutikazon sometimes showed the efficiency at pulmonary I-III stages whereas combinations of system and inhalation steroids positively influenced both clinical symptomatology, and changes on roentgenograms at the II-IV stages. Besides, early appointment in Prednisolonum (20 mg/days for 3 months) with the subsequent transition to inhalations of a budesonid within 15 months (800 mkg a day) is followed by the smaller frequency of aggravations and the best pulmonary function in comparison with placebo.

At extra pulmonary defeat usually use Prednisolonum in a dose of 0,5-1,0 mg/kg/days with the subsequent decrease when signs of activity of a disease decrease. Opinions on the level of a maintenance therapy and its duration are contradictory; the dosage varies depending on the struck body. Thus, at damage of kidneys the initial dose of Prednisolonum usually makes 1 mg/kg/days with the supporting level of 5-10 mg of Prednisolonum not less than during 1 year. If the disease does not give in to adequate control, then use drugs of the second line, such as Azathioprinum.