Fiore Nicoletta, Castellano Giuseppe, Blasi Antonella, Capobianco Carmen, Loverre Antonia, Montinaro Vincenzo, Netti Stefano, Torres Diletta, Manno Carlo, Grandaliano Giuseppe, Ranieri Elena, Schena Francesco Paolo, Gesualdo Loreto
Renal Unit, Department of Emergency and Organ Transplantation, University of Bari, Policlinico, Piazza G. Cesare 11, 70124 Bari, Italy.
Mol Immunol. 2008 Jan;45(1):259-65. doi: 10.1016/j.molimm.2007.04.029. Epub 2007 Jun 13.
Systemic lupus erythematosus (SLE) is an autoimmune disease involving several organs. SLE patients developing lupus nephritis (LN) frequently have the worst outcome. Recent data have shown that dendritic cells (DCs) may have a central role in SLE pathogenesis directing the immune response against auto-antigens. In this study we describe a reduction in circulating BDCA1+ and BDCA3+ myeloid DCs, and BDCA2+ plasmacytoid DCs in patients with active LN compared to those in the remission state. Analysis of LN biopsies revealed a strong tubulo-interstitial infiltrate of BDCA1+, BDCA3+ and BDCA4+ DCs which were negative for DC-LAMP, a specific marker of mature DCs. The extent of the DCs infiltrate was higher in class III/IV LN than in normal kidney. These results show for the first time that three DCs subsets, decreased at circulating levels, are recruited within the kidney, indicating that DCs might play a pathogenic role in SLE patients with nephritis.
系统性红斑狼疮（SLE）是一种累及多个器官的自身免疫性疾病。发生狼疮性肾炎（LN）的SLE患者通常预后最差。最近的数据表明，树突状细胞（DCs）可能在SLE发病机制中发挥核心作用，引导针对自身抗原的免疫反应。在本研究中，我们描述了与缓解期患者相比，活动性LN患者循环中BDCA1 +和BDCA3 +髓样DCs以及BDCA2 +浆细胞样DCs减少。对LN活检组织的分析显示，BDCA1 +、BDCA3 +和BDCA4 + DCs在肾小管间质有强烈浸润，而这些细胞对成熟DCs的特异性标志物DC-LAMP呈阴性。III/IV级LN中DCs浸润程度高于正常肾脏。这些结果首次表明，循环水平降低的三种DCs亚群被募集到肾脏内，表明DCs可能在患有肾炎的SLE患者中发挥致病作用。
