ISSN 1006-298X      CN 32-1425/R

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肾脏病与透析肾移植杂志 ›› 2017, Vol. 26 ›› Issue (1): 72-75.DOI: 10.3969/cndt.j.issn.1006-298X.2017.01.016

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狼疮足细胞病:一种特殊类型的狼疮性肾炎

  

  • 出版日期:2017-02-28 发布日期:2017-02-21

Lupus podocytopathy: a distinct entity of lupus nephritis

  • Online:2017-02-28 Published:2017-02-21

摘要:

系统性红斑狼疮患者临床表现为肾病综合征,肾活检病理表现为肾小球轻微病变(MCD)、系膜增生(MsP)或局灶节段性肾小球硬化(FSGS),超微结构以足细胞足突广泛融合为特征,毛细血管袢内皮下及上皮侧无免疫沉积物的肾损害称为狼疮足细胞病。肾小球病理改变为MCD和MsP的狼疮足细胞病患者,血尿发生率低,激素治疗敏感,但单用激素维持的复发率高达90%,激素联合其他免疫抑制剂维持可显著降低复发率。而病理表现为FSGS的狼疮足细胞病患者,急性肾损伤的发生率高,肾小管间质损伤重,激素治疗缓解率低。狼疮足细胞病复发后可发生病理转型,远期预后良好。狼疮足细胞病应作为一类特殊类型狼疮性肾炎纳入新的病理分型体系中。

关键词: 狼疮性肾炎, 狼疮足细胞, 病病理分型

Abstract:

SLE patients clinically presenting with nephrotic syndrome demonstrated minimal change disease (MCD), mesangial hypercellularity (MsP) or focal segmental glomerulosclerosis (FSGS); on electronic microscopy, diffuse effacement of the podocyte foot processes was the single morphologic feature in the absence of subepithelial or subendothelial electron dense deposits, which was called as lupus podocytopathy. Lupus podocytopathy with glumerular change of MCD or MsP presented with a low rate of hematuria, and sensitive to glucocorticoid. In maintenance treatment, the relapse rate of glococorticoid monotherapy reached up to 90%, and glococorticoid plus other immunosuppressive agents could significantly lower the rate of relapse. While patients with FSGS suffered a higher rate of acute kidney injure and lower rate of remission to glucocorticoid. Longterm outcomes of these patients were optimistic, but pathological transition occurred after relapse. The unique clinicalpathological features indicated lupus podocytopathy should be classified as a special subtype and enrolled in the current pathological classification of lupus nephritis.

Key words: lupus nephritis, lupus podocyto, pathypathological classification