Ⅳ型狼疮性肾炎不同病理亚型的临床病理与预后

Differences between Class ⅣS and ⅣG in patients with lupus nephritis

目的:探讨弥漫增生性狼疮性肾炎不同病理亚型[弥漫性节段型(ⅣS型)和球型(ⅣG型)]患者临床病理特点及预后。方法:157例经肾活检病理明确诊断的Ⅳ型LN患者,根据肾小球病理改变分为ⅣS组(n=42)和ⅣG组(n=115)。诱导治疗采用激素联合间断环磷酰胺静脉冲击治疗(IVCY)或激素联合吗替麦考酚酯(MMF)。回顾性分析两组患者临床、病理及预后的差异。结果:ⅣS组和ⅣG组基线肾脏损害指标无差异。ⅣS组血清补体C3(0.493 g/L vs 0.355 g/L,P<0.01)和C4水平(0.111 g/L vs 0.065 g/L,P<0.01)均显著高于ⅣG组,补体C4降低发生率明显低于ⅣG组(50.0%vs 80.9%,P<0.01)。肾活检组织学观察证实,ⅣS组白金耳(0vs 42.6%,P<0.01)和微血栓(2.4%vs 36.5%,P<0.01)比例显著低于ⅣG组。ⅣS组患者6个月完全缓解(CR)率显著低于ⅣG组(33.3%vs 53.0%,P=0.029)。其中,ⅣS+IVCY组6个月CR率显著低于ⅣG+IVCY组(18.8%vs 52.7%,P=0.016)。ⅣS组10年无血清肌酐倍增或进展至终末期肾病患者生存率显著低于ⅣG组(70.4%vs 97.1%,P=0.015)。结论:ⅣS型LN肾小球呈现"寡免疫复合物沉积"特点,传统免疫抑制治疗的缓解率及肾脏远期预后均较ⅣG型LN差,提示Ⅳ型LN不同亚型可能存在不同的发病机制不同。

Objective：This study aimed to determine the differences in clinicopathological features and outcome between segmental and global LN. Methodology：Forty-two patients with biopsy-proven segmental LN （Class IV S group） and 115 with global LN （Class IV G group） were identified and retrospectively analyzed. Classification criteria were modified in our study. Class IV S LN was defined as t〉 50% of glomeruli manifesting crescents or fibrinoid necrosis, and the uninvolved glomerular and capillary loop were normal. Class IVG LN was defined as 〉150% of the glomeruli manifested as endocapillary proliferation involving the whole glomerular without fibrinoid necrosis or crescent. Results： （ 1 ） Class IV S had significantly higher complement C3 （ 0. 493 vs 0. 355 g/L, P〈 0.01 ） and Cd （ 0. 111 vs 0. 065 g/L, P〈 0. 01 ） than Class IVG. The proportion of low complement C4 in IV S group was significantly lower than in IV G group （50. 0% vs 80.9%,P〈0.01）.（2） Wire loop（0% vs 42.6%,P〈0.01） and hyaline thrombi（2.4% vs 36.5%,P〈0.01） were significantly rare in IV S than in IV G group. （3）The complete remission （CR） rate within 6 months was significantly lower in the IVS（33.3% vs 53.0% ,P=0. 029） than in IVG group. CR rate within 6 months of IVCY＋IVS group（ 18.8% vs 52. 7% ,P= 0. 016） was significantly lower than that of IVCY＋ IV G group. The 10-year survival without renal function deterioration in IV S group was significantly inferior than that in IV G group （70.4% vs 97.1%,P = 0. 015）. Conclusion： Compared to global LN, segmental LN demonstrated a ＂panic-immune-complex deposit＂ pathology, a dissatisfied response to traditional immunosuppressant and a poorer prognosis, suggesting different pathogenesis may exsit.