The weak stability between immune competence and immune tolerance is mirrored within the coexistence, in the identical Inborn Error of Immunity (IEI), of susceptibility to infections and predisposition to develop immune dysregulatory options. Totally different mechanisms are implicated within the pathogenesis of immunodeficiency and autoimmunity. The thymus performs an important position within the improvement of each immune tolerance and immune competence. Alterations of thymus improvement and performance could result in IEIs presenting with a large spectrum of dysregulation of the immune system starting from severely poor T-cell operate, as in extreme mixed immunodeficiency, to severely dysregulated T-cell operate, as in Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy (APECED).
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