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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: To investigate the clinicopathological features and prognosis of diffuse proliferative lupus nephritis with different pathological subtypes (diffuse segmental type ⅣS and type ⅣG). Methods: A total of 157 patients with type Ⅳ LN diagnosed by renal biopsy were divided into ⅣS group (n = 42) and ⅣG group (n = 115) according to the pathological changes of glomerulus. Induction therapy with hormone combined with intermittent cyclophosphamide intravenous therapy (IVCY) or hormone combined with mycophenolate mofetil (MMF). The clinical, pathological and prognostic differences between the two groups were retrospectively analyzed. Results: There was no difference in baseline renal damage between ⅣS and ⅣG groups. Serum complement C3 (0.493 g / L vs 0.355 g / L, P <0.01) and C4 (0.111 g / L vs 0.065 g / L, P <0.01) in ⅣS group were significantly higher than those in ⅣG group Which was significantly lower than that of ⅣG group (50.0% vs 80.9%, P <0.01). Histological examination of renal biopsies confirmed that the proportion of platinum-ear (0 vs 42.6%, P <0.01) and microthrombus (2.4% vs 36.5%, P <0.01) in ⅣS group was significantly lower than that in ⅣG group. The complete remission (CR) rate of patients in ⅣS group was significantly lower than that in ⅣG group at 6 months (33.3% vs 53.0%, P = 0.029). Among them, the CR rate of 6 months in IVS + IVCY group was significantly lower than IVG + IVCY group (18.8% vs 52.7%, P = 0.016). The survival rates of serum creatinine doubling or progression to end-stage renal disease in IVS group were significantly lower than those in IVG group (70.4% vs 97.1%, P = 0.015). Conclusion: The glomeruli of type ⅣS LN presented the characteristics of “oligo-immune complex deposition”. The remission rate of traditional immunosuppressive therapy and the long-term renal prognosis were both worse than that of ⅣG type LN, suggesting that different subtypes of type Ⅳ LN may have different Different pathogenesis.