Targeted therapy for systemic sclerosis

摘要: Systemic sclerosis is a multisystem disease whose therapy focused at pathogenic pathways causing variable types of damage in the individual organs. There are 3 major that cause organ scleroderma. First, t-cells, cytokines and inflammation prominent very early disease. Early alveolitis which occurs before interestial fibrosis lungs best example inflammation. Second, endothelial cell causes severe thickening vessels two most deadly complications scleroderma, pulmonary arterial hypertension renal crisis. Scleroderma crisis now treatable with angiotensin converting enzyme inhibitors. treatments for should improve outcome these patients as well. Third, fibroblasts lead to cutaneous or skin hallmark No treatment available but we hopeful new antagonists cytokine, TGF beta, will prove helpful. B cells autoantibodies not involved pathogenesis disease, there scleroderma specific antibodies help defining patient subsets. All factors influenced by unknown inciting agents permissive genetic background.