Ubara Yoshifumi, Sawa Naoki, Wada Takehiko, Kono Kei, Ohashi Kenichi
Nephrology Center and Okinaka Memorial Institute for Medical Research, Toranomon Hospital, 2-2-2, Toranomon, Minato, Tokyo, Japan.
Department of Pathology, Toranomon Hospital, Tokyo, Japan.
Clin Exp Nephrol. 2025 May;29(5):507-520. doi: 10.1007/s10157-024-02603-6. Epub 2024 Dec 19.
Renal lesions due to systemic lupus erythematosus (SLE) are defined as lupus nephritis (LN), a renal disease characterized by the deposition of immunoglobulin (Ig)G-based immune complexes in the kidney and the appearance of double-stranded DNA and Smith antibodies. In particular, deposition of IgG3, which has strong complement binding properties, under the endothelium or in the mesangium activates the classical complement pathway of C1q, C4, and C3, leading to renal damage. This step is followed by migration of inflammatory cells, including neutrophils and monocytes, which induce inflammation in the glomerular capillaries and cause mesangiolysis and endothelial cell damage, resulting in endocapillary proliferative nephritis. LN is classified as class I to IV depending on the degree of inflammation or as class V in cases of subepithelial deposition of immune complexes in glomeruli. Deposition in the renal small arterioles by the same mechanism induces thrombus formation, resulting in lupus vasculopathy. Deposition in the tubular basement membrane and peritubular capillaries leads to tubulointerstitial lupus nephropathy. The appearance of antiphospholipid antibodies leads to acute and chronic forms of antiphospholipid antibody nephropathy (APSN) due to thrombus formation. This article reviews cases of the typical diverse renal lesions in LN.
系统性红斑狼疮(SLE)所致的肾脏病变被定义为狼疮性肾炎(LN),这是一种以基于免疫球蛋白(Ig)G的免疫复合物在肾脏沉积以及双链DNA和史密斯抗体出现为特征的肾脏疾病。特别是具有强补体结合特性的IgG3在内皮细胞下或系膜中的沉积会激活C1q、C4和C3的经典补体途径,导致肾脏损伤。这一步之后是炎症细胞(包括中性粒细胞和单核细胞)的迁移,这些细胞会在肾小球毛细血管中引发炎症,导致系膜溶解和内皮细胞损伤,从而引起毛细血管内增生性肾炎。LN根据炎症程度分为I至IV级,若肾小球中免疫复合物在上皮下沉积则归为V级。通过相同机制在肾小动脉中的沉积会诱导血栓形成,并导致狼疮性血管病变。在肾小管基底膜和肾小管周围毛细血管中的沉积会导致肾小管间质性狼疮性肾炎。抗磷脂抗体的出现会因血栓形成导致急性和慢性形式的抗磷脂抗体肾病(APSN)。本文回顾了LN中典型多样的肾脏病变病例。