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• W2064581943 abstract "Cytokines are criticalmediators of the immune response. This review focuses on cytokine-specific information from children with juvenile dermatomyositis, and includes pertinent data from adults with polymyositis and dermatomyositis.Much of the new data concern the role of possible antigens and the definition of genetic control of the immune response in juvenile dermatomyositis. Gene expression profile data of DQA1*0501 (present in 85% of patients) compared with age-matched control subjects show that the initial immune response is an interferon-alpha/beta-induced cascade with secondary stimulation of interferon-gamma. Specific epitopes of group A beta-hemolytic streptococcal M protein, with sequence homology for myosin, elicit both cell-mediated cytotoxicity and tumor necrosis factor-alpha production when incubated with mononuclear cells from children with active juvenile dermatomyositis. Tumor necrosis factor-alpha synthesis is increased in juvenile dermatomyositis patients with the tumor necrosis factor-alpha-308A allele, and is associated with increased thrombospondin-1 (an antiangiogenic agent) production and small vessel occlusion in untreated juvenile dermatomyositis. Studies in adults with polymyositis and dermatomyositis implicate interleukin-1alpha, transforming growth factor-beta, and endothelial cell perturbation early in the disease course. Cultured myoblasts were found to produce interleukin-15, which impacts local T-cell activation and proliferation.The limited data suggest that a possible viral/microbial antigen may elicit an interferon-alpha/beta-induced response, and that antigenic epitopes may be shared. Increased synthesis of tumor necrosis factor-alpha, more common in juvenile dermatomyositis with the tumor necrosis factor-alpha-308A polymorphism, may augment this response and is associated with a wide range of pathologic consequences, as well as disease chronicity and calcifications. The muscle fibers themselves can regulate local inflammation by production of tumor necrosis factor-alpha, interleukin-15 and interleukin-1alpha, and transforming growth factor-beta." @default.
• W2064581943 created "2016-06-24" @default.
• W2064581943 creator A5005206087 @default.
• W2064581943 creator A5041302124 @default.
• W2064581943 date "2003-11-01" @default.
• W2064581943 modified "2023-09-26" @default.
• W2064581943 title "Cytokines in juvenile dermatomyositis pathophysiology: potential and challenge" @default.
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• W2064581943 doi "https://doi.org/10.1097/00002281-200311000-00003" @default.
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