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斯耶格伦氏(Sjogren)综合征(SS)是以干燥性角膜炎、慢性唾液腺炎为主要特征的原因不明的自身免疫性疾病。病理组织学显示以泪腺、唾液腺等导管及腺泡周围显著的淋巴细胞浸润为特征,尔后呈现腺破坏、萎缩所致的干燥症。因为病变发生于外分泌腺泡系统,故称为自身免疫性外分泌病。本病表现显著的高γ球蛋白血症,存在多克隆的B细胞活化。在一部分病例,发生从低克隆到单克隆B细胞株增殖致进行性B细胞淋巴瘤。由此可见,SS是处于自身免疫病与恶性肿瘤之间的特殊性疾病。据1976年厚生省调查研究组调查,SS的发病率
Sjogren’s syndrome (SS) is an unexplained autoimmune disease characterized by dry keratitis and chronic salivary gland inflammation. Histopathology showed that lacrimal gland, salivary gland and other ductal and acinar lymphocytes around the significant infiltration was characterized by gland destruction, shrinkage caused by xerostomia. Because lesions occur in the exocrine alveolar system, it is called autoimmune exocrine disease. The disease showed significant hypergammaglobulinemia, polyclonal B cell activation. In some cases, progressive B-cell lymphoma occurs as a result of the proliferation of monoclonal B cell lines from low clones. Thus, SS is a particular disease between autoimmune diseases and malignant tumors. According to the survey conducted by the Ministry of Health, Labor and Welfare in 1976, the incidence of SS was investigated