IMMUNE ABNORMALITIES IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS

SLE患者B细胞过度增殖。他们已经增加了 骨髓干细胞和循环B细胞前体细胞的增殖。 疾病活动的特征是从仅仅扩散到 分化和免疫球蛋白合成。在某些患者中，T细胞 B细胞功能调节异常。为了更好地 了解细胞间的相互作用，研究了正态分布的容量 T细胞对自体非T细胞的反应。T4生产IL-2 正性细胞是T8plus(抑制子)增殖所必需的 细胞。在系统性红斑狼疮患者中，这种功能是不正常的。 单抗用于研究SLE患者的亚群。 其中一个子集，辅助细胞/抑制细胞的比率降低 以肾脏疾病、血小板减少、白细胞减少和 发病年龄早。另一个子集具有高比率和 临床表现包括肌肉和肺部疾病，西卡综合征， 淋巴结病和中枢神经系统疾病。因此，系统性红斑狼疮可能不是单一的疾病，但 一组具有不同遗传和细胞基础的综合征。 SLE患者存在多种T细胞缺陷。这些在很大程度上得到了解释 由这些患者的B细胞产生的抗T细胞抗体。 进展越快的自身免疫性淋巴或增殖性疾病， 血管免疫母细胞性淋巴结病的特点是显著有效 辅助T细胞，驱动B细胞增殖和分化。B类 细胞本身在这个变种中是正常的。 SLE患者原癌基因表达异常。有一个 活化的B细胞的表达特性显著增强。

Patients with SLE have excessive B cell proliferation. They have increased proliferation of bone marrow stem cells and circulating B cell precursors. Disease activity is characterized by a switch from just proliferation to differentiation and immunoglobulin synthesis. In some patients the T cell regulation of B cell functions is abnormal. In an attempt to better understand cell-cell inter-actions, normals were studied for the capacity of T cells to respond to autologous non-T cells. IL 2 production by T4 plus cells was necessary for the proliferation of T8 plus (suppressor) cells. This function is subnormal in patients with SLE. Monoclonal antibodies were used to study subpopulations of SLE patients. One subset, with a reduced ratio of helper/suppressor cells, was characterized clinically by renal disease, thrombocytopenia, leukopenia and early age of onset of disease. Another subset had a high ratio and a clinical picture which included muscle and lung disease, Sicca syndrome, lympadenopathy and CNS disease. Thus, SLE may not be a single disease, but a group of syndromes with different genetic and cellular bases. SLE patients have a variety of T cell defects. These are largely explained by anti-T cell antibodies produced by the B cells of these patients. The more rapidly progressive autoimmune lymphorproliferative disorder, angioimmunoblastic lymphadenopathy is characterized by markedly effective helper T cells which drive B cells to proliferate and differentiate. The B cells themselves are normal in this varient. Proto-oncogene expression is abnormal in patients with SLE. There is a significant increase in expression characteristic of activated B cells.