Systemic lupus erythematosus and the type I interferon system.
Systemic lupus erythematosus and the type I interferon system.
复制标题
DOI：
10.1186/ar625
复制
发表时间：
2003
影响因子：
4.9
通讯作者：
Alm GV
中科院分区：
文献类型：
--
作者：
Rönnblom L;Alm GV
系统性红斑狼疮（SLE）患者持续产生干扰素-α (IFN-α)，血清IFN-α水平与疾病活动性和严重程度相关。最近对SLE患者的研究表明，在这些个体中存在内源性IFN-α诱导剂，由含有IgG和DNA的小免疫复合物（ic）组成。这些ic特异性作用于天然IFN-α-产生细胞（NIPCs），通常被称为浆细胞样树突状细胞（PDCs）。考虑到NIPC/PDC在先天和适应性免疫反应中都起关键作用，以及IFN-α的许多免疫调节作用，这些观察结果可能对了解SLE的发病机制很重要。在这篇综述中，我们简要地描述了I型IFN系统的生物学，重点是诱导剂、产生细胞（特别是NIPCs/PDCs）、IFN-α的作用和可能与SLE相关的靶免疫细胞。基于这些信息和对SLE患者的研究结果，我们提出了一个假说来解释NIPCs/PDCs如何被激活并在SLE中起关键的致病作用。这一假设也为这种自身免疫性疾病提供了新的治疗靶点。
Patients with systemic lupus erythematosus (SLE) have ongoing interferon-α (IFN-α) production and serum IFN-α levels are correlated with both disease activity and severity. Recent studies of patients with SLE have demonstrated the presence of endogenous IFN-α inducers in such individuals, consisting of small immune complexes (ICs) containing IgG and DNA. These ICs act specifically on natural IFN-α-producing cells (NIPCs), often termed plasmacytoid dendritic cells (PDCs). Given the fact that the NIPC/PDC has a key role in both the innate and adaptive immune response, as well as the many immunoregulatory effects of IFN-α, these observations might be important for the understanding of the etiopathogenesis of SLE. In this review we briefly describe the biology of the type I IFN system, with emphasis on inducers, producing cells (especially NIPCs/PDCs), IFN-α actions and target immune cells that might be relevant in SLE. On the basis of this information and results from studies in SLE patients, we propose a hypothesis that explains how NIPCs/PDCs become activated and have a pivotal etiopathogenic role in SLE. This hypothesis also indicates new therapeutic targets in this autoimmune disease.
登录
查看更多内容
影响因子：
30.5
作者：
Cella, M;Facchetti, F;Colonna, M
通讯作者：
Colonna, M
影响因子：
12.8
作者：
Cederblad, B;Blomberg, S;Ronnblom, L
通讯作者：
Ronnblom, L
影响因子：
4.4
作者：
Cocca, BA;Cline, AM;Radic, MZ
通讯作者：
Radic, MZ
影响因子：
3.8
作者：
Chuntharapai, A;Lai, J;Stewart, TA
通讯作者：
Stewart, TA
影响因子：
2.6
作者：
Bengtsson, AA;Sturfelt, G;Rönnblom, L
通讯作者：
Rönnblom, L