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IgM肾病:临床表现与长期预后

IgM nephropathy: clinical picture and long-term prognosis.

作者信息

Myllymäki Juhani, Saha Heikki, Mustonen Jukka, Helin Heikki, Pasternack Amos

机构信息

Medical School, University of Tampere; and Departments of Medicine and Pathology, Tampere University Hospital, Tampere, Finland.

出版信息

Am J Kidney Dis. 2003 Feb;41(2):343-50. doi: 10.1053/ajkd.2003.50042.

Abstract

BACKGROUND

Immunoglobulin M (IgM) nephropathy is an idiopathic glomerulonephritis with mesangial hypercellularity and diffuse IgM deposits.

METHODS

We studied clinical presentation, morphological findings, and prognostic factors in 110 patients with IgM nephropathy without systemic diseases. The series included both pediatric and adult patients with nephrotic syndrome (NS) or minor urinary abnormalities.

RESULTS

Mean postbiopsy follow-up was 8 years. During 15 years of follow-up, 36% of patients developed renal insufficiency and 23% reached end-stage renal failure. In multivariate analysis, hypertension at the time of renal biopsy was the only significant risk factor for renal insufficiency. Of histological parameters, interstitial fibrosis had the strongest prognostic value. Hypertension was diagnosed in 50% of patients with a postbiopsy follow-up of 15 years. Twenty-nine percent of nephrotic patients had disease resistant to corticosteroids, whereas 80% of patients with steroid-sensitive disease were steroid dependent. Eleven patients, 8 patients with NS and 3 patients with asymptomatic proteinuria, underwent repeated renal biopsy. In five samples, typical morphological characteristics of focal and segmental glomerulosclerosis were seen.

CONCLUSION

We propose that IgM nephropathy can be divided into two subgroups with similar renal biopsy findings, but differences in sex distribution and initial presentation.

摘要

背景

免疫球蛋白M(IgM)肾病是一种特发性肾小球肾炎,具有系膜细胞增多和弥漫性IgM沉积。

方法

我们研究了110例无全身性疾病的IgM肾病患者的临床表现、形态学发现及预后因素。该系列包括患有肾病综合征(NS)或轻度尿液异常的儿童和成人患者。

结果

活检后平均随访8年。在15年的随访期间,36%的患者出现肾功能不全,23%的患者达到终末期肾衰竭。多因素分析显示,肾活检时的高血压是肾功能不全的唯一显著危险因素。在组织学参数中,间质纤维化具有最强的预后价值。在活检后随访15年的患者中,50%被诊断为高血压。29%的肾病患者对糖皮质激素耐药,而80%对激素敏感的患者依赖激素。11例患者,8例患有NS,3例患有无症状蛋白尿,接受了重复肾活检。在五个样本中,可见局灶节段性肾小球硬化的典型形态学特征。

结论

我们提出IgM肾病可分为两个亚组,肾活检结果相似,但性别分布和初始表现存在差异。

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