SARKOIDOZ

SARKOIDOZ (syn: Bénia-Beka-Schaumann disease) is a systemic disease with a chronic course characterized by the formation of specific granulomas in various organs and tissues. Sarcoid granulomas differ from tuberculosis mainly by the absence of necrotic changes. In affected organs, the development of fibrosis, leading to a persistent disruption of their function, is possible. According to modern ideas, sarcoidosis is a disease of impaired immunoreactivity with a special reaction of the body to the effect of various environmental factors. It occurs at the age of 20 - 50 years, more often women are sick.

In most cases, the intrathoracic lymph nodes, bronchi and lungs are affected (sarcoidosis of the respiratory system); Combined defeat of two or three organs is possible, a generalized form of sarcoidosis involving many organs and tissues, an extrathoracic form of sarcoidosis.

Sarcoidosis of the respiratory system usually begins asymptomatically and is detected accidentally by radiographic examination of the lungs. As the disease develops, a dry cough appears, sometimes pain in the chest, then the leading one becomes a complaint of shortness of breath at first with a high, then with normal physical activity. Hard breathing and dry wheezes can be detected. X-ray examination allows to detect an increase in the intrathoracic lymph nodes (mainly bilateral), focal shadows in the lungs, diffuse pulmonary infiltration, pleural reaction. With a long progressing course of the process, lung fibrosis is formed, the vital capacity of the lungs decreases, respiratory failure increases (dyspnea occurs even at rest, diffuse cyanosis appears, which increases during exercise); It is often possible to identify a symptom of the tympanic fingers. Dyspnea in sarcoidosis of the lungs is inspiratory (hampered, limited in the volume of inhalation, free rapid exhalation), the speech of the patients is intermittent due to the need to often replenish the exhaled volume of air.

High position of the lower border of the lungs and restriction of their respiratory excursions are determined percussionally. Above the lower and middle sections of the lungs are often listened to homogeneous in the caliber and sonority of the wet rales of a crackling timbre. Gradually, a picture of the chronic pulmonary heart is formed, which decompensates in the late stages of the disease.

For the generalized form of sarcoidosis, complaints of weakness, fatigue, decreased appetite, loss of weight, pain in the joints. There are signs of damage to organs and tissues, for example, enlargement of the liver, peripheral lymph nodes, radiographic examination - changes in the organs of the chest cavity, etc .; Is possible subfebrile condition. In some cases, an acute onset of the disease with fever is observed, nodosum erythema appears, pain in the muscles and joints (sometimes polyarthritis is possible).

Of the extrahuman manifestations, sarcoidosis of the skin, sarcoidosis hepatitis (without significant violations of the liver functions), spleen involvement, peripheral lymph nodes (in most cases cervical, which are enlarged but painless and not soldered to surrounding tissues), skeletal bones (diffuse osteoporosis, cystic Changes more often in the distal phalanges of fingers and feet), the eye (sarcoidosis iridocyclitis involving the retina and the choroid of the eye, sometimes the optic nerve). Less common sarcoidosis is myocarditis (manifested by arrhythmias and rapidly developing heart failure), meningitis and meningoencephalitis (severe, usually fatal), neuritis (often the facial nerve).

In sarcoidosis of the skin, mostly small (1-5 mm in diameter) or a few large nodules (sarcoids of the skin) or indistinctly outlined brownish-cyanotic foci (diffuse-focussing) are formed mainly on the face, upper half of the trunk, extensor surfaces of the upper limbs and the back of the fingers of the hands. Infiltrative sarcoid). Characteristic are small yellow spots in the center of nodules or infiltrative foci when they are squeezed by a transparent plate (for example, slide), superficial skin atrophy in place of disappearing nodules with pigmentation (often in the form of a rim) or depigmentation and the formation of telangiectasias. A variety of sarcoidosis of the skin is lupus erythematosus, or an enlarged luposarcoid, in which fusiform vesicles sometimes appear on the fingers of the hands (radiographically, the cystic ostitis of phalanges is detected), blistering rashes can easily form on the affected areas, which easily heal superficial ulceration. Sometimes there are atypical manifestations of sarcoidosis of the skin, reminiscent of its changes with red flat lishy, ​​erythroderma, etc.

In the course of sarcoidosis, the phases of exacerbation and remission are usually observed. During the exacerbation of the disease, general weakness develops, pains in the muscles and joints occur; Increased ESR, observed leukocytopenia, lymphocytopenia, monocytosis. As a manifestation of hypercalcemia, thirst, polyuria , nausea , constipation are possible. The most serious complications of sarcoidosis are respiratory failure and pulmonary heart disease in sarcoidosis of the respiratory system; Glaucoma , cataract , persistent loss of vision in sarcoidosis of the eye.

Treatment in the active phase consists in the long-term (6 months or more) use of glucocorticoids inside or locally (for example, with eye damage). They must be prescribed to patients with lesions of the eyes, heart, nervous system, with generalized sarcoidosis. In complex treatment, immunosuppressants, delagil, and vitamin E are also used. Depending on the localization of the lesion and the nature of the complications, appropriate pathogenetic and symptomatic therapy is performed (treatment of respiratory failure, uncompensated pulmonary heart, surgical treatment of complications of eye sarcoidosis, etc.).

The prognosis for life and ability to work in the early stages of treatment is favorable. Possible recovery after the first course of hormone therapy; A number of patients achieve long-term remission. In the late stages of the disease, treatment is ineffective. Patients with stable respiratory failure and pulmonary heart are not able to work; In this stage of sarcoidosis of the respiratory system, as well as in sarcoidosis of the heart and the central nervous system, the prognosis for life is significantly deteriorating.

Prevention, aimed at preventing exacerbations and complications of the disease, consists in the timely conduct of treatment courses based on the results of dynamic monitoring of patients who are subject to medical examination in anti-tuberculosis facilities.