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进行性系统性硬化症(PSS)存在多种免疫异常,它们有助于诊断,但尚不清楚起什么样的病因学作用。也就是说尚不能肯定PSS 是否真正是自身免疫性疾病。然而,根据 PSS 存在抗核抗体等多种自身抗体,淋巴细胞功能异常,以及可与 SLE、干燥综合征等自身免疫病重叠等,一般认为其属于自身免疫性疾病范畴。特别,已发现 PSS 存在特异性的免疫异常,其重要性正在增加。阐明这些免疫异常与疾病发生之间的关系是今后的重要研究课题。
Progressive systemic sclerosis (PSS) There are a variety of immune abnormalities, they contribute to the diagnosis, but it is not clear what kind of etiology. That is to say it is not yet certain whether PSS is really an autoimmune disease. However, PSS is thought to belong to the autoimmune disease category due to the presence of various autoantibodies such as antinuclear antibodies, abnormal lymphocyte function, overlapping with autoimmune diseases such as SLE and Sjogren’s syndrome. In particular, PSS has been found to have specific immunological abnormalities that are of increasing importance. To clarify the relationship between these immune disorders and the occurrence of the disease is an important research topic in the future.