弥漫性节段性和单纯弥漫性全球增殖性肾小球肾炎：IV 型狼疮性肾炎的不同模式。

Diffuse segmental and pure diffuse global proliferative glomerulonephritis: different patterns of class IV lupus nephritis.

作者信息

Xu Shengchun, Liu Zhengzhao, Chen Huiping, Zeng Caihong, Zhang Haitao, Liu Zhihong, Hu Weixin

机构信息

Nanjing University School of Medicine, National Clinical Research Center of Kidney Diseases, Jinling Hospital, Nanjing, China.

出版信息

Clin Nephrol. 2014 Jun;81(6):411-8. doi: 10.5414/cn108191.

DOI:10.5414/cn108191

PMID:25017669

Abstract

BACKGROUND

No consensus has been obtained on the differences between class IV-S and IV-G lupus nephritis (LN), especially regarding renal outcome. Our study investigated clinical-pathological features and prognosis of diffuse segmental and pure diffuse global proliferative LN.

METHODS

In this retrospective study, a total of 120 patients with biopsy-proven diffuse LN were included, of which 31 patients were class IV-S and 89 were pure class IVG. Class IV-S was defined as segmental lesion involving ≥ 50% of all glomeruli, while pure class IV-G was defined as global lesion involving ≥ 50% of all glomeruli with no segmental necrosis or crescents. The clinical- pathological and prognostic features of the two classes were compared.

RESULTS

There was no difference in levels of urine protein or serum creatinine between the two groups. Higher serological activity was observed in the pure IV-G group with lower complement C3 (p < 0.001) and C4 level (p < 0.001), compared to the IV-S group. Histologically, immune-complex deposits were significantly more common in the pure IV-G group, with higher prevalence of wire loop (42.7% vs. 0%, p < 0.001) and hyaline thrombi (34.8% vs. 3.2%, p < 0.001). However, the complete remission (CR) rate to intravenous cyclophosphamide (IVCY) induction was lower in the IV-S than in the pure IV-G group (16.7% vs. 53.2%, p = 0.023). After 1 year, the pure class IV-G group had a higher CR rate (71.9% vs. 48.4%, p = 0.017). The 10-year renal survival rate (without doubling of serum creatinine or end-stage renal disease) was significantly lower in patients with IV-S than pure IV-G (75.2% vs. 97.4%, p = 0.028).

CONCLUSION

LN class IV-S and class IV-G without segmental lesion showed different clinical-histological features and prognosis, suggesting that different mechanisms may exist.

摘要

背景

关于IV - S级和IV - G级狼疮性肾炎（LN）之间的差异，尤其是在肾脏预后方面，尚未达成共识。我们的研究调查了弥漫性节段性和单纯弥漫性球性增殖性LN的临床病理特征和预后。

方法

在这项回顾性研究中，共纳入120例经活检证实为弥漫性LN的患者，其中31例为IV - S级，89例为单纯IV - G级。IV - S级定义为节段性病变累及所有肾小球的≥50%，而单纯IV - G级定义为球性病变累及所有肾小球的≥50%，且无节段性坏死或新月体形成。比较了两组的临床病理和预后特征。

结果

两组间尿蛋白水平或血清肌酐水平无差异。与IV - S组相比，单纯IV - G组血清学活动度更高，补体C3水平更低（p < 0.001），C4水平更低（p < 0.001）。组织学上，单纯IV - G组免疫复合物沉积明显更常见，白金耳（42.7%对0%，p < 0.001）和透明血栓（34.8%对3.2%，p < 0.001）的发生率更高。然而，IV - S组对静脉环磷酰胺（IVCY）诱导的完全缓解（CR）率低于单纯IV - G组（16.7%对53.2%，p = 0.023）。1年后，单纯IV - G组的CR率更高（71.9%对48.4%，p = 0.017）。IV - S级患者的10年肾脏生存率（血清肌酐未翻倍或未达到终末期肾病）显著低于单纯IV - G级患者（75.2%对97.4%，p = 0.028）。