[Pathogenesis of systemic scleroderma: immunological aspects].

摘要: Systemic sclerosis (SSc) is a connective tissue disorder that characterized by excessive collagen synthesis fibroblasts and vascular hyperreactivity obliteration phenomena. Excessive production the consequence of abnormal interactions between endothelial cells, mononuclear cells. Immunological abnormalities are present very early in development SSc. Mononuclear particularily macrophages T lymphocytes play prominent role fibroblast activation through cytokines they produce. Thus, lymphocytic infiltrates skin lung preferentially composed CD8+ lymphocytes, produce important amounts interleukin 4 (IL-4). The effects IL-4 added to these transforming growth factor B (TGF-B) (CTGF) stimulate fibroblasts. gamma interferon (INF-gamma) best inhibitor However, inhibitory effect INF-gamma on diminished SSc patients. Numerous autoantibodies can be evidenced serum Three them specific for mutually exclusive: anti-centromere antibodies (Ab) limited SSc, anti-Scl70 Ab diffuse anti-RNA polymerase III with renal involvement. These good prognosis markers but their pathogenic remains uncertain.