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肾脏病与透析肾移植杂志 ›› 2015, Vol. 24 ›› Issue (5): 401-406.

• 论文 •    下一篇

微小病变样IgA肾病与微小病变肾病患者临床病理及预后的比较

  

  • 出版日期:2015-10-28 发布日期:2015-10-30

The comparison of clinico-pathological features and outcome between IgA nephropathy with minimal change disease and minimal change disease

  • Online:2015-10-28 Published:2015-10-30

摘要:

摘要 目的:比较微小病变样IgA肾病(MCD-IgAN)与微小病变肾病(MCD)患者临床病理特点、治疗反应及预后异同。方法:回顾性分析IgAN随访登记数据库中随访时间≥3年的80例MCD-IgAN和随访时间≥3年的77例MCD患者资料。结果:(1)临床表现上两组患者相似,以青年男性多见,多数患者以肾病综合症为主要表现,部分患者伴不同程度的镜下血尿,但与MCD患者相比,MCD-IgAN患者肾活检时基线血清白蛋白、eGFR更低(P均<0.01),而尿NAG、尿RBP水平更高(P均<0.01)。(2)肾脏病理:MCD-IgAN与MCD患者相比,肾小管间质急性病变重(P<0.01),小管间质慢性病变也较重(P<0.05)。(3)激素疗效: 80例MCD-IgAN与77例MCD患者激素治疗的疗效比较,8周完全缓解、部分缓解及无效均无显著差异(88.8% vs 90.9%, 10.0% vs 5.2%, 1.3% vs 3.9%, P均>0.05)。达到缓解的中位时间分别是4周(范围1~24周)和4周(范围1~28周)。两组患者激素疗效无显著差异(P>0.05)。(4)复发和预后:至3年随访期末, MCD-IgAN患者复发率显著低于MCD患者(45.0% vs 63.6%, P<0.05).没有患者进入ESRD,仅有2例MCD-IgAN患者eGFR较基线下降大于50%。结论:MCD-IgAN与MCD患者在临床表现,病理特点、激素治疗的疗效及预后上均无明显差异。值得注意的是,MCD-IgAN患者伴较显著的肾小管间质病变和损伤。

关键词: IgA肾病, 微小病变肾病, 临床病理, 治疗, 预后

Abstract:

 ABSTRACT Objective: To investigate the clinic-pathological characteristics, treatment response and prognosis of IgA nephropathy patients with minimal change lesion (MCD-IgAN). Methodology: Eighty patients with biopsy-proven MCD-IgAN and followed for ≥3 years from the Jinling Hospital IgA nephropathy Registry were enrolled into this retrospective study, and 77 patients with MCD were regarded as disease control. The clinic-pathological characteristics, treatment response and prognosis were compared with between two groups. Results: The clinical presentations were similar between the two groups, both with young male predominance, the majority of these patients presented with nephrotic syndrome, only few MCD-IgAN had varying degrees of macroscopic hematuria. Compared with the MCD group, patients with MCD-IgAN had lower levels of baseline serum albumin (P <0.01) and eGFR (P<0.01), higher levels of urinary n-acetyglucosaminidase and retino-bingding protein (P <0.01). The pathological features showed more severe acute and chronic tubulointerstitial injury in patients with MCD-IgANrenal pathology (P <0.01, P <0.05, respectively), while the glomerular lesion was similar between two groups. After 8 weeks of corticosteroid therapy, no significant differences were observed in the rate of complete remission, partial remission and no remission between MCD-IgAN and MCD (88.8% vs 90.9%, 10.0% vs 5.2%, 1.3% vs 3.9%, P>0.05). The median time to achieve remission was 4 weeks (range 1-24 weeks) and 4 weeks (range 1-28 weeks) respectively. During the three-year follow-up period, there was a significantly lower rate of relapse in MCD-IgAN patients than that in MCD patients (45.0% vs 63.6%, P <0.05). No patients between two groups entered ESRD, only 2 patients (2.5%) with MCD-IgAN had greater than 50% reduction of eGFR. Conclusion: The clinical manifestations, pathological features, response to corticosteroid therapy and renal outcome were similar between MCD-IgAN and MCD.

Key words: IgA nephropathy, minimal change disease, clinic-pathological features, treatment, prognosis