Pathogenesis of Systemic Sclerosis.

摘要: Systemic scleroderma (SSc) is one of the most complex systemic autoimmune diseases. It targets vasculature, connective tissue-producing cells (namely fibroblasts/myofibroblasts), and components innate adaptive immune systems. Clinical pathologic manifestations SSc are result of: (1) innate/adaptive system abnormalities leading to production autoantibodies cell-mediated autoimmunity, (2) microvascular endothelial cell/small vessel fibroproliferative vasculopathy, (3) fibroblast dysfunction generating excessive accumulation collagen other matrix in skin internal organs. All three these processes interact affect each other. The disease heterogeneous its clinical presentation that likely reflects different genetic or triggering factor (i.e., infection environmental toxin) influences on system, tissue cells. roles played by ubiquitous molecular entities (such as lysophospholipids, endocannabinoids, their diverse receptors vitamin D) influencing just beginning be realized studied may provide insights into new therapeutic approaches treat SSc.