Schur P H
Department of Rheumatology/Immunology, Brigham and Women's Hospital, Harvard Medical School, Boston Mass.
Nephrologie. 1988;9(2):53-60.
SLE is characterized by a host of immune abnormalities. It is not clear to date which of these are primary and which are secondary. The observation of a number of genetic defects suggests that they are primary. Multiple genetic defects may then lead to abnormal immune responses to common pathogens, antigens, or even autoantigens. As a result of this abnormal immune response, immune complexes form with resultant complement fixation and activation. These immune complexes interacting with cells and complement initiate an inflammatory response. One can also speculate that this inflammatory response represents a normal response to an abnormal event, or is also abnormal in the SLE patient. The ultimate result is tissue inflammation and often damage. While at present our therapy is aimed at controlling these secondary inflammatory phenomena mediated by immune complexes and complement, ultimately therapy may be more successful after the primary defects are corrected.
系统性红斑狼疮的特征是一系列免疫异常。至今尚不清楚其中哪些是原发性的，哪些是继发性的。对一些基因缺陷的观察表明它们是原发性的。多种基因缺陷可能会导致对常见病原体、抗原甚至自身抗原产生异常免疫反应。这种异常免疫反应的结果是形成免疫复合物，并导致补体固定和激活。这些免疫复合物与细胞和补体相互作用引发炎症反应。人们也可以推测，这种炎症反应代表了对异常事件的正常反应，或者在系统性红斑狼疮患者中也是异常的。最终结果是组织炎症，常常伴有组织损伤。虽然目前我们的治疗旨在控制由免疫复合物和补体介导的这些继发性炎症现象，但最终在纠正原发性缺陷后治疗可能会更成功。
