Tsokos G C, Kovacs B, Liossis S N
Department of Clinical Physiology, Walter Reed Army Institute of Research, Washington, DC 20307-5100, USA.
Curr Opin Rheumatol. 1997 Sep;9(5):380-6. doi: 10.1097/00002281-199709000-00002.
Studies reported during the past year have added new knowledge to our understanding of the pathogenesis of systemic lupus erythematosus. A study of sibpairs with lupus revealed a strong linkage of a region located at chromosome 1q41-42 that crossed ethnic barriers. B-cell receptor-initiated signaling events, such as tyrosine protein phosphorylation and intracellular calcium concentrations, were found to be increased in patients with lupus in a disease- and clinical activity-independent manner. T cells from patients with lupus express increased amounts of the CD40 ligand, which is functional because it helps B cells to produce anti-DNA antibodies and express more CD80 (B7-1) on their surface. Only occasionally do lupus patients display structural defects of either Fas antigen or ligand molecules, and although spontaneous apoptosis is increased in lupus cells (as well as in other systemic autoimmune disorders), the activation-induced T-cell death is defective.
过去一年中发表的研究为我们对系统性红斑狼疮发病机制的理解增添了新知识。一项针对狼疮患者同胞对的研究发现，位于1号染色体1q41 - 42区域存在强烈连锁，且跨越了种族界限。狼疮患者中，B细胞受体启动的信号事件，如酪氨酸蛋白磷酸化和细胞内钙浓度，以与疾病及临床活动无关的方式增加。狼疮患者的T细胞表达量增加的CD40配体具有功能，因为它有助于B细胞产生抗DNA抗体并在其表面表达更多的CD80（B7 - 1）。狼疮患者仅偶尔出现Fas抗原或配体分子的结构缺陷，尽管狼疮细胞（以及其他系统性自身免疫性疾病）的自发凋亡增加，但激活诱导的T细胞死亡存在缺陷。
