返回搜索

Systemic lupus erythematosus

KEGG ID: hsa05322

中文名称

系统性红斑狼疮

通路描述

系统性红斑狼疮(SLE)是一种典型的自身免疫性疾病,其特征是产生针对自身抗原(如 DNA、核蛋白和某些细胞质成分)的特异性 IgG 自身抗体,并伴随多种临床表型。SLE 患者主要的病理发现是炎症、血管炎、免疫复合物沉积和血管病。由自身抗体和自身抗原组成的免疫复合物特异性沉积于肾小球,并通过激活补体或 FcγR 介导的中性粒细胞和巨噬细胞活化,引发全身性炎症反应。补体(C5)的激活会导致损伤,一方面通过形成膜攻击复合物(C5b-9),另一方面通过生成过敏毒素和细胞激活物 C5a。中性粒细胞和巨噬细胞通过释放氧化剂和蛋白酶导致组织损伤。
英文描述
Systemic lupus erythematosus (SLE) is a prototypic autoimmune disease characterised by the production of IgG autoantibodies that are specific for self-antigens, such as DNA, nuclear proteins and certain cytoplasmic components, in association with a diverse array of clinical manifestations. The primary pathological findings in patients with SLE are those of inflammation, vasculitis, immune complex deposition, and vasculopathy. Immune complexes comprising autoantibody and self-antigen is deposited particulary in the renal glomeruli and mediate a systemic inflammatory response by activating complement or via Fc{gamma}R-mediated neutrophil and macrophage activation. Activation of complement (C5) leads to injury both through formation of the membrane attack complex (C5b-9) or by generation of the anaphylatoxin and cell activator C5a. Neutrophils and macrophages cause tissue injury by the release of oxidants and proteases.

所含基因

141 个基因