Advances in pathogenesis and treatment of systemic sclerosis
Advances in pathogenesis and treatment of systemic sclerosis
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DOI：
10.7861/clinmedicine.16-1-55
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发表时间：
2016-02-01
影响因子：
4.4
通讯作者：
Denton, Christopher P.
中科院分区：
文献类型：
--
作者：
Denton, Christopher P.
系统性硬化症是硬皮病谱系中最严重的疾病，是一个具有高死亡率和发病率的主要医学挑战。在了解反映免疫炎症过程与血管病变和纤维化之间相互作用的发病机制方面取得了进展。它可以被认为是一种结缔组织修复疾病，并导致器官并发症。然而，病因和触发事件仍有待阐明。这种疾病的许多方面都有治疗方法，但现有的治疗方法不能治愈，一些并发症仍然非常具有挑战性，特别是非致命的表现，如疲劳、钙质沉着症和肛肠功能障碍。免疫抑制现在被认为是一种有益的方法，但平衡风险和收益是至关重要的，特别是对于自体干细胞移植等强大的方法。
Systemic sclerosis is the most severe disease within the scleroderma spectrum and is a major medical challenge with high mortality and morbidity. There have been advances in understanding of pathogenesis that reflect the interplay between immune-inflammatory processes and vasculopathy and fibrosis. It can be regarded as a disease of connective tissue repair and this leads to organ-based complications. However the aetiology and triggering events remain to be elucidated. Treatment is available for many aspects of the disease although the available therapies are not curative and some complications remain very challenging, especially non-lethal manifestations such as fatigue, calcinosis and anorectal dysfunction. Immunosuppression is now established as a beneficial approach but balancing risk and benefit is vital, especially for powerful approaches such as autologous stem cell transplantation.