Mageed R A, Prud'homme G J
Department of Immunology and Molecular Pathology, Royal Free and University College School of Medicine, London, UK.
Gene Ther. 2003 May;10(10):861-74. doi: 10.1038/sj.gt.3302016.
Lupus is a chronic autoimmune inflammatory disease with complex clinical manifestations. In humans, lupus, also known as systemic lupus erythematosus (SLE), affects between 40 and 250 individuals, mostly females, in each 100 000 of the population. There are also a number of murine models of lupus widely used in studies of the genetics, immunopathology, and treatment of lupus. Human patients and murine models of lupus manifest a wide range of immunological abnormalities. The most pervasive of these are: (1) the ability to produce pathogenic autoantibodies; (2) lack of T- and B-lymphocyte regulation; and (3) defective clearance of autoantigens and immune complexes. This article briefly reviews immunological abnormalities and disease mechanisms characteristic of lupus autoimmunity and highlight recent studies on the use of gene therapy to target these abnormalities.
狼疮是一种临床表现复杂的慢性自身免疫性炎症性疾病。在人类中,狼疮,也称为系统性红斑狼疮(SLE),每10万人中约有40至250人受其影响,其中大多数为女性。还有许多狼疮小鼠模型广泛应用于狼疮遗传学、免疫病理学及治疗的研究。狼疮患者和狼疮小鼠模型表现出广泛的免疫异常。其中最普遍的是:(1)产生致病性自身抗体的能力;(2)T淋巴细胞和B淋巴细胞调节功能缺失;(3)自身抗原和免疫复合物清除缺陷。本文简要综述了狼疮自身免疫的免疫异常和疾病机制,并重点介绍了针对这些异常进行基因治疗的最新研究。