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Systemic sclerosis – multidisciplinary disease: clinical features and treatment

机译:系统性硬化症-多学科疾病:临床特征和治疗

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摘要

Systemic sclerosis is a chronic autoimmune disease of still not fully understood pathogenesis. Fibrosis, vascular wall damage, and disturbances of innate and acquired immune responses with autoantibody production are prominent features. Systemic sclerosis has specific subsets with different autoantibodies, and differences in the affected skin areas. The suspicion of systemic sclerosis and establishing the diagnosis will be facilitated by the criteria created by EULAR/ACR experts. The treatment of this autoimmune disease remains a challenge for clinicians and new therapeutic options are constantly sought. The occurrence of various symptoms and the involvement of many organs and systems make systemic sclerosis a multidisciplinary disease and require a holistic approach. The present article summarizes different clinical features of systemic sclerosis and the profile of autoantibodies and discusses recent rules and future perspectives in disease management.
机译:系统性硬化症是一种慢性自身免疫性疾病,其发病机理尚未完全明了。纤维化,血管壁损伤以及自身抗体产生对先天和后天免疫反应的干扰是突出特征。系统性硬化症具有特定的亚群,具有不同的自身抗体,并且受影响的皮肤区域也不同。 EULAR / ACR专家创建的标准将有助于怀疑系统性硬化症并确定诊断。这种自身免疫疾病的治疗仍然是临床医生面临的挑战,并且不断寻求新的治疗选择。各种症状的发生以及许多器官和系统的参与使系统性硬化症成为多学科疾病,需要采取整体方法。本文总结了全身性硬化症的不同临床特征和自身抗体的概况,并讨论了疾病管理中的最新规则和未来观点。

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