系统性硬化新进展

Progress in systemic sclerosis

系统性硬化(systemic sclerosis,SSc)是一种原因不明的复杂的系统性结缔组织病,系统性硬化有多种亚型,它们的临床表现和预后各不相同。虽然已表明SSc发病主要是由于血管炎和纤维化异常,应用血管抑制制剂可以缓解诸如硬皮病肾危象、继发肺动脉高压等病情,近几年随着对SSc发病机制的进一步研究,发现了一些治疗新视点,本文就SSc这些新进展做一综述。

Systemic sclerosis（SSc）is a complex and chronic multisystem autoimmune disease in clinical manifestations that has multiple overlapping and poorly defined clinical subsets.internal organ involvement,and outcome.The pathogenesis of SSc encompasses vascular immunological and fibrotic processes,which contribute clinical manifestations and morbidity and must be addressed in the treatment plan.Although vascular interventions appear to reduce the frequency and severity of complications,such as scleroderma renal crisis and pulmonary hypertension current therapies generally target the immune component of SSc in a non-selective fashion and have largely failed a diseases-modifying interventions.Newer insights into the mechanisms underlying autoimmunity,vascular injury and destruction,and particularly tissue fibrosis provide novel potential targets for therapy.This is an update about SSc.