固有免疫应答在系统性硬皮病发病机制中的研究进展

The recent progress of innate immunity in the pathogenesis of systemic sclerosis

系统性硬皮病(systemic sclerosis,SSc)是以皮肤及各内脏器官慢性纤维化为特征的自身免疫病。SSc发病中,固有免疫应答在激活免疫系统和纤维化过程中均发挥重要作用。固有免疫系统一方面通过抗原提呈作用,建立与适应性免疫应答的桥梁;另一方面通过分泌和调节关键的细胞因子促进炎症反应和组织纤维化过程。本文针对固有免疫系统中关键的单核/巨噬细胞、肥大细胞、中性粒细胞和树突状细胞等细胞成分,以及近年来重点关注的Toll样受体及其配体和IL-4、IL-6、TGF-β等细胞因子进行综述,旨在阐明固有免疫应答在SSc发病中的作用,以助于本病的临床评估及靶向分子治疗。

Systemic sclerosis(SSc)is an autoimmune disease characterized by progressive fibrosis of the skin and various internal organs.The innate immunity is crucial in the activation of inflammatory response and onset of fibrosis in the pathogenesis of SSc.On one hand,the innate immune system regulates the activation of adaptive immune system through antigen presentation,and on the other hand,it promotes local inflammation and matrix accumulation via production of various proinflammatory cytokines.This review aims at the important role of monocytes/macrophages,mast cells,neutrophils and dendritic cells in innate immune system,as well as Toll-like receptors and their ligands and proinflammatory cytokines(including IL-4,IL-6 and TGF-β)in SSc.The elaboration of innate immunity in the pathogenesis of SSc will contribute to the clinical implications and the therapeutic targeting of effector molecules.