Scleroderma Edema

Topic: "Edematous scleroderma" *Introduction*

Scleroderma edematous (hereinafter referred to as ES) is one of the most common systemic connective tissue diseases, characterized by progressive diffuse thickening of the skin and internal organs due to dysplastic changes in the connective tissue. The basis of this disease is progressive genetic instability and increased susceptibility to cytokines, in particular the cytokine TNF-α. OM manifests itself with various symptoms, including fever, myalgia, arthralgia, and changes in the gastrointestinal tract. This syndrome belongs to the group of multifactorial diseases and is considered as a sign of a systemic inflammatory response caused by sensitization of T or B lymphocytes to circulating antigens. The main cause of the development of the disease is exposure to toxins, infectious factors, and waste products of microorganisms. Thus, the relevance of studying this pathology is associated with the ambiguity of understanding the etiology and pathogenesis, the lack of effective treatment methods and the high risk of developing lethal complications. This work is devoted to a description of the clinical manifestations of edematous scleroderma and an analysis of methods for diagnosing and treating this disease.

*Pathogenesis*

Clinical manifestations of OM are associated with damage to the connective tissue of the skin and internal organs. These pathomorphological studies confirm the formation of connective tissue deformations against the background of hyperproliferation of fibroblasts and abnormal production of the extracellular matrix. Genetic risk factors are carriage of the HLA-DRB1\*11 and HLA-B51 alleles. It has been established that in OM, the level of interleukins IL-1, IL-6, TNF, interferon gamma IFN-γ, tumor necrosis factor TNF-α, antibodies to interferon α2b IFNα2b is increased, and activation of immune system cells, inflammation of adipose tissue (pulmonary artery and its branches). These patients are characterized by a high incidence of erythema migrans with changes in the muscles of the heart, lungs and limbs. Myositis develops, manifested by muscle pain and wasting caused by dysfunction of the immune system. The functional state of the central and peripheral nervous system is disrupted, degenerative changes develop in the cartilaginous tissue of the bronchial