Denton Christopher P
Royal Free Hospital and UCL Division of Medicine, London, UK.
Clin Med (Lond). 2015 Dec;15 Suppl 6:s58-63. doi: 10.7861/clinmedicine.15-6-s58.
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.
系统性硬化症是硬皮病谱系中最严重的疾病，是一项具有高死亡率和发病率的重大医学挑战。在发病机制的理解方面已有进展，这反映了免疫炎症过程、血管病变和纤维化之间的相互作用。它可被视为一种结缔组织修复疾病，这会导致基于器官的并发症。然而，病因和触发事件仍有待阐明。针对该疾病的许多方面都有治疗方法，尽管现有疗法无法治愈，且一些并发症仍然极具挑战性，尤其是疲劳、钙质沉着和肛门直肠功能障碍等非致命性表现。免疫抑制现已被确立为一种有益的方法，但平衡风险和益处至关重要，尤其是对于自体干细胞移植等强效方法。
