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目的:探讨狼疮性肾炎(LN)是否存在肾小管管周毛细血管(PTC)C4d沉积以及其沉积的病理生理机制。方法:455例经肾活检证实的LN患者,用间接免疫荧光法检测肾活检冰冻切片组织C4d、C1q和C3的沉积,并与21例C4d阳性的移植肾急性排斥患者进行比较。结果:15例LN患者(3·29%)肾小管PTCC4d弥漫阳性主要为Ⅳ型狼疮,其中,Ⅱ型LN3例,Ⅳ型LN9例,Ⅲ+Ⅳ型LN1例,Ⅳ+Ⅴ型LN2例。本组患者抗核抗体均强阳性,73·3%的患者抗dsDNA阳性,40%的患者抗Smith抗体阳性,60%的患者抗心磷脂抗体阳性,所有患者补体C3均明显下降,80%患者补体C4下降。同时,LNPTCC4d沉积阳性的患者,绝大多数合并其他补体成分的沉积,12例(80%)患者伴有C1q共沉积,4例患者(26·7%)伴C3沉积,2例患者(13·3%)伴IgG沉积,而21例移植肾C4d阳性急性排斥患者未发现其他补体成分的共沉积。LN患者PTCC4d阳性沉积者肾组织PTC无中性粒细胞浸润。结论:部分LN患者肾组织PTCC4d沉积,其存在自身免疫功能亢进和补体经典途径的激活。与移植肾急性排斥反应伴PTCC4d阳性的沉积者不同,LN患者PTCC4d阳性的同时,合并有其他补体成分的沉积,而其PTC并无特殊的病理学改变,提示LN患者PTCC4d沉积的机制有别于抗体介导的肾移植急性排斥,它的存在可能代表了LN中一个相对独特的亚型。
Objective: To investigate the pathophysiological mechanism of C4d deposition and deposition of peritubular capillaries (PTC) in lupus nephritis (LN). Methods: 455 LN patients confirmed by renal biopsy were used to detect the deposition of C4d, C1q and C3 in the frozen section of renal biopsy by indirect immunofluorescence and compared with 21 cases of acute rejection of C4d positive renal allograft. Results: Among 15 LN patients (3.29%), diffuse positive of tubular PTCC4d was mainly type Ⅳ lupus. Among them, type Ⅱ LN3 cases, type Ⅳ LN9 cases, type Ⅲ + Ⅳ LN1 cases and type Ⅳ + Ⅴ LN2 cases. Anti-nuclear antibodies were strongly positive in this group of patients, anti-dsDNA positive in 73.3% of patients, anti-Smith antibodies in 40% of patients, anti-cardiolipin antibodies in 60% of patients, all patients with C3 were significantly decreased, 80% Complement C4 decreased. At the same time, the majority of LNPTCC4dpositive patients, most of them with other components of complement deposition, 12 patients (80%) with C1q co-deposition, 4 patients (26.7%) with C3 deposition, 2 patients 3%) with IgG deposition, and 21 cases of grafted C4d positive acute rejection patients found no co-deposition of other complement components. LN patients with PTCC4d-positive deposits of renal tissue PTC no neutrophil infiltration. Conclusion: The deposition of PTCC4d in renal tissue of some patients with LN has autoimmune hypersensitivity and activation of classical complement pathway. In contrast to the acute rejection of PTCs and the positive deposits of PTCC4d, LN patients with positive PTCC4d and other complement components were deposited with no specific pathological changes of PTC, suggesting that the mechanism of PTCC4d deposition in LN patients differs from that of LN patients Antibody-mediated acute rejection of renal transplantation, whose presence may represent a relatively distinct subtype in LN.