systemic sclerodermia

Sklerodermia System (CDW) Autoimmunnoe disease connective tissue, the main manifestation of which related to the disease, and fibrosis of organs and tissues. Etiology disease is not known. A surplus develops under the influence of some external factors in people with certain genetic disorders. The exogenous factors capable induced development of SMEs are retrovirusa (primarily zitomegalovirusa), quartz and coal dust, organic solvents exist, some medicines (bleomycin, and a number of other drugs used for chemotherapy). AA economies is a combination of many factors, which play a key role immune activation, vascular damage endotelia and improve the synthetic fibroblasts. The severity of each of these factors varies pathogenesis of individual patients. As systemic disease economies characterized by the simultaneous loss of skin, vessels, musculoskeletal system and internal organs, including heart, lungs, kidneys and stomach. The inaugural debut until specific symptoms often constitutional manifestations : loss of weight subfebrilnaya fever, weakness. Has two main clinical forms CDW-and limited in diffuznuu. Limitirovannaya form characterized by the following features : Reynaud syndrome for many years before the emergence of other symptoms; Skin lesion confined to the field of individuals and distal extremities; Later developed pulmonary hypertension with / without interstitial lung fibrosis; High frequency identification anticentromernykh antibodies (from 70 to 80% of patients); Expansion capillaries without significant awasculiarnah sites. Jimmy form has its own characteristics : the development of skin changes in the first year after the syndrome Reynaud; Skin involvement of all divisions of limbs and trunk; Palpathornoe identification friction tendons; Early development of interstitial fibrosis of the lungs, stomach damage, and kidney diseases; And the expansion of direct capillaries; Antibody topoizomeraze-1 (Scl-70) and Rnk-polimerazam. A correlation between the existence of systemic sclerodermia and development of malignant tumors. In patients with ASEAN likely future development of lung cancer, 5%; Skin-4%; Liver-3%; Hemoblastosis-2%; Breast cancer and ovarian cancers. Risk Development oncopatologii higher for patients with diffuse form (breast cancer, bladder, lungs).