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免疫复合物介导的膜增生性肾小球肾炎与 C3 肾小球病的诊断和预后比较。

Diagnostic and Prognostic Comparison of Immune-Complex-Mediated Membranoproliferative Glomerulonephritis and C3 Glomerulopathy.

机构信息

Department of Pathology, University of Helsinki and Helsinki University Hospital, 00290 Helsinki, Finland.

Department of Nephrology, University of Helsinki and Helsinki University Hospital, 00290 Helsinki, Finland.

出版信息

Cells. 2023 Feb 23;12(5):712. doi: 10.3390/cells12050712.

Abstract

Membranoproliferative glomerulonephritis (MPGN) is subdivided into immune-complex-mediated glomerulonephritis (IC-MPGN) and C3 glomerulopathy (C3G). Classically, MPGN has a membranoproliferative-type pattern, but other morphologies have also been described depending on the time course and phase of the disease. Our aim was to explore whether the two diseases are truly different, or merely represent the same disease process. All 60 eligible adult MPGN patients diagnosed between 2006 and 2017 in the Helsinki University Hospital district, Finland, were reviewed retrospectively and asked for a follow-up outpatient visit for extensive laboratory analyses. Thirty-seven (62%) had IC-MPGN and 23 (38%) C3G (including one patient with dense deposit disease, DDD). EGFR was below normal (≤60 mL/min/1.73 m) in 67% of the entire study population, 58% had nephrotic range proteinuria, and a significant proportion had paraproteins in their serum or urine. A classical MPGN-type pattern was seen in only 34% of the whole study population and histological features were similarly distributed. Treatments at baseline or during follow-up did not differ between the groups, nor were there significant differences observed in complement activity or component levels at the follow-up visit. The risk of end-stage kidney disease and survival probability were similar in the groups. IC-MPGN and C3G have surprisingly similar characteristics, kidney and overall survival, which suggests that the current subdivision of MPGN does not add substantial clinical value to the assessment of renal prognosis. The high proportion of paraproteins in patient sera or in urine suggests their involvement in disease development.

摘要

膜增生性肾小球肾炎(MPGN)分为免疫复合物介导的肾小球肾炎(IC-MPGN)和 C3 肾小球病(C3G)。经典的 MPGN 具有膜增生性模式,但根据疾病的时间过程和阶段,也描述了其他形态。我们的目的是探讨这两种疾病是否真的不同,还是仅仅代表相同的疾病过程。回顾性分析了芬兰赫尔辛基大学医院区 2006 年至 2017 年间诊断的 60 例符合条件的成人 MPGN 患者,并邀请他们进行随访门诊,进行广泛的实验室分析。37 例(62%)为 IC-MPGN,23 例(38%)为 C3G(包括 1 例致密沉积物病,DDD)。整个研究人群中,67%的 EGFR 低于正常水平(≤60 mL/min/1.73 m),58%有肾病范围蛋白尿,相当一部分患者血清或尿液中有副蛋白。整个研究人群中仅 34%表现为经典 MPGN 模式,组织学特征分布相似。基线或随访期间的治疗在两组之间没有差异,随访时补体活性或成分水平也没有显著差异。两组的终末期肾病风险和生存率相似。IC-MPGN 和 C3G 具有惊人相似的特征,包括肾脏和总体生存率,这表明 MPGN 的当前细分并未为评估肾脏预后提供实质性的临床价值。患者血清或尿液中存在大量副蛋白提示其参与疾病的发展。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/caa5/10000503/65742819d272/cells-12-00712-g001.jpg

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