van Paassen P, Tervaert J W Cohen, Heeringa P
Department of Clinical and Experimental Immunology, Cardiovascular Research Institute Maastricht, University Maastricht, Maastricht, The Netherlands.
Nephron Exp Nephrol. 2007;105(1):e10-6. doi: 10.1159/000096960.
Both the innate and the acquired immune system are involved in the pathophysiology of renal vasculitis. However, anti-neutrophil cytoplasmic antibody (ANCA)-associated renal vasculitis is characterized by a 'pauci-immune' pattern of immunofluorescence during kidney biopsy, indicating the relative lack of immunoglobulin and complement deposition within the kidney. On the other hand, evidence is accumulating that ANCA, autoantibodies against constituents of primary granules of neutrophils and the lysosomes of monocytes, play a pathogenic role in renal vasculitis. In this review we will discuss both in vitro and in vivo experimental data providing compelling evidence that ANCA are a primary pathogenic factor in renal vasculitis, mainly by augmenting leukocyte-endothelial interactions. We will also address novel data, pointing at the role of, in addition to ANCA, non-specific proinflammatory signals. Finally, we propose a working hypothesis of the pathogenesis of ANCA-associated renal vasculitis.
先天性和获得性免疫系统均参与肾血管炎的病理生理学过程。然而，抗中性粒细胞胞浆抗体（ANCA）相关的肾血管炎在肾脏活检时的免疫荧光表现为“少免疫”模式，这表明肾脏内免疫球蛋白和补体沉积相对较少。另一方面，越来越多的证据表明，ANCA（针对中性粒细胞初级颗粒成分和单核细胞溶酶体的自身抗体）在肾血管炎中起致病作用。在本综述中，我们将讨论体外和体内实验数据，这些数据提供了令人信服的证据，表明ANCA主要通过增强白细胞与内皮细胞的相互作用，是肾血管炎的主要致病因素。我们还将探讨新的数据，这些数据指出除了ANCA之外，非特异性促炎信号也发挥作用。最后，我们提出了ANCA相关肾血管炎发病机制的工作假说。
