Scleroderma Systemic

Sclerosing therapy in patients with systemic scleroderma can be used both in routine clinical practice and for the treatment of severe emergency conditions - acute systemic necrosis of the skin and subcutaneous tissue. However, due to the high toxicity of steroids, their use may be limited - for example, in terms of potentiation of additive side effects (for example, gastrointestinal disorders) when associated with infections or without the availability of other effective therapy in patients requiring high doses of intravenous glucocorticoids, especially in emergency treatment conditions. It is important to emphasize that glucocorticoids administered into the vascular bed cause systemic hormonal disorders (hypothalamic-pituitary-adrenal collapse) without a specific effect on the inflammatory process. In this sense, it is clear that the administration of high doses of glucocorticoids with a long T2 must be carefully documented due to their toxic consequences on the patient's immune system. In many cases, patients with systemic sclerosis, who are in a severe, potentially life-threatening condition, require alternative, more gentle regimens of drug pathogenetic therapy, based on suppression of the inflammatory response, which do not lead to systemic hormonal disorders. Such a therapeutic alternative today is genetically engineered biological drugs and other monoclonal antibodies (ATs), which are effective both in systemic skin lesions and in increasing disabling systemic arthritis. This therapeutic approach implies an effective local cytoprotective effect, optimization of metabolic processes, restoration of the normal architecture of collagen fibers without interfering with the pituitary-adenopituitary hormonal system. It is also important to suppress the activation of the immune system under the influence of the anti-inflammatory background and inflammatory mediators (IL4, IL6).

Thus, the results of the prospective study **FASCIAL-T**, which included 43 patients with familial (non-inflammatory) systemic sclerosis (SSc), characterized by generalized pemphigoid skin lesions with severe sclerosis, are of interest. Patients (33 women and 10 men) received a tumor necrosis factor (TNF) antagonist drug, a monoclonal antibody to TNF, which completely reproduces the molecular structure of this cytokine (Illumina, registration certificate No. LP-002989 dated February 22, 2015). Patients with SSc maintained the ability to suppress TNF during long-term treatment with a TNF antagonist. The most pronounced microcirculatory and histological signs of SSc were identified in the group of patients who did not receive a TNF antagonist (p = 0.037), while by the 42nd day of treatment, 8 weeks from the start of therapy, the differences between the groups were leveled (p = 1.0) . Patients with SSc who received a monoclonal antibody to tumor necrosis factor showed a noticeable decrease in skin thermal indices.

Scleroderma (erythematoscledera, sclero-eratadermia) is a chronic multifactorial systemic inflammatory disease of connective tissue of unknown etiology, occurring with a predominance of proliferative and vascular disorders and ending in skin sclerosis. Sometimes this term is used to refer to general swelling and infiltration of subcutaneous fat without disruption of skin function. Synonyms. Systemic rheumatic diseases: dermatosis from the group of sclerosing skin diseases (sclerodinia), limited (diffuse) scleroderma dermatitis, lupoderma, leukodystrophic (pasty) skin, subepidermal panniculitis, panniculitis-like dystrophy. The pathogenesis of the disease is caused by a combination of hyperproduction of various cytokines by T-lymphocytes (including tumor necrosis factor alpha) with the formation of autoantibodies to the filaggrin system, which depolymerizes under the influence of phlogogens, which leads to the destruction of elastic fibers of the skin - Raynaud's syndrome. The course is pallidal and recurrent. It is characterized by successive phases of pronounced inflammatory erythema (vascular phase), edema resulting from hyperemia and hyperpigmentation of the skin, as well as atrophic changes in the skin: pigmentation like “apple jelly” or ivory spots, impaired thermoregulation in the form of induration (increased tissue density as a result of impaired trophism). Characteristic are hanging and dense nodules (in the first months) or plaques (late stages of the disease). Subjectively, both forms manifest themselves as stiffness, soreness (painful “cotton-wool” bump) and/or debilitating throbbing pain (acute stage); thickening, decreased functionality and/or generalization of skin