Center of Research of Immunopathology and Rare Diseases - Coordinating Center of the Network for Rare Diseases of Piedmont and Aosta Valley, Department of Clinical and Biological Sciences and University of Turin, Italy; SCU Nephrology and Dialysis Unit, S. Giovanni Bosco Hospital and University of Turin, Turin, Italy.
Rheumatology Unit, University Hospital, Padua, Italy.
Autoimmun Rev. 2017 Oct;16(10):1024-1028. doi: 10.1016/j.autrev.2017.07.018. Epub 2017 Aug 1.
Systemic sclerosis (SSc) or scleroderma is a disorder of the connective tissue affecting the skin, and it is often associated with visceral involvement. The predominant pathological features of SSc are autoimmunity, vasculopathy, and fibrosis. Progressive fibrosis is associated with changes in the microcirculation of the involved organs. Here, we review the clinical features of systemic sclerosis and describe the best practice approaches for its management, reviewing available guidelines and recommendations and providing experts' insights.
系统性硬化症(SSc)或硬皮病是一种影响皮肤的结缔组织疾病,常伴有内脏受累。SSc 的主要病理特征是自身免疫、血管病变和纤维化。进行性纤维化与受累器官微循环的变化有关。在这里,我们回顾了系统性硬化症的临床特征,并描述了其管理的最佳实践方法,回顾了现有的指南和建议,并提供了专家的见解。