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伴有和不伴有微小病变病的轻微系膜增生性 IgA 肾病。

Mild mesangial proliferative IgA nephropathy with and without minimal change disease.

机构信息

Department of Nephrology, The First Affiliated Hospital of Zhengzhou University, No. 1 Jianshe East Road, Zhengzhou, Henan, People's Republic of China.

Research Institute of Nephrology, Zhengzhou University, Zhengzhou, Henan, People's Republic of China.

出版信息

Clin Exp Med. 2023 Dec;23(8):5367-5376. doi: 10.1007/s10238-023-01184-0. Epub 2023 Oct 5.

DOI:10.1007/s10238-023-01184-0
PMID:37796359
Abstract

Mild mesangial proliferative IgA nephropathy with minimal change disease (MCD-IgAN) and mild mesangial proliferative IgA nephropathy without minimal change disease (Non-MCD-IgAN) have similar characteristics on light microscopy. Nevertheless, their discrepancies in clinicopathological features and prognosis remain unknown. A total of 589 patients with biopsy-proven mild mesangial proliferative IgA nephropathy (M-IgAN) combined with light microscopy and immunofluorescence were enrolled. Firstly, the diagnoses of the patients by electron microscopy were recorded and used as the gold standard. We calculated the sensitivity and specificity using nephrotic syndrome (NS) as the diagnostic criteria to identify MCD-IgAN. Then, excluding patients with a 24-h urinary total protein less than 0.5 g/day, incomplete clinical data, or less than the six-month follow-up, we included 184 cases of non-MCD-IgAN and 98 cases of MCD-IgAN. The patients' clinicopathological and outcome data were collected and compared. Among the 589 patients, according to electron microscopy, 381 were diagnosed with non-MCD-IgAN, 167 with MCD-IgAN, and 41 with M-IgAN complicated by other glomerular diseases. Using NS as the diagnostic criteria to distinguish non-MCD-IgAN and MCD-IgAN, the sensitivity and specificity were 83.8% and 99.5%, respectively. The patients in the MCD-IgAN group tended to be younger, hypotensive, with lower urinary erythrocytes, and more likely to achieve complete remission, and fewer patients progressed to the endpoint than those in the non-MCD-IgAN group (all P < 0 .05). NS appears to be an objective indicator for differentiating MCD-IgAN from non-MCD-IgAN. Non-MCD-IgAN varies greatly from MCD-IgAN in clinicopathology and treatment response, with a poorer prognosis.

摘要

轻度系膜增生性 IgA 肾病伴微小病变病(MCD-IgAN)和不伴微小病变病的轻度系膜增生性 IgA 肾病(Non-MCD-IgAN)在光镜下具有相似的特征。然而,它们在临床病理特征和预后方面的差异尚不清楚。共纳入 589 例经活检证实的轻度系膜增生性 IgA 肾病(M-IgAN)患者,结合光镜和免疫荧光检查。首先,以电镜诊断为金标准,记录患者的诊断。我们以肾病综合征(NS)为诊断标准,计算 MCD-IgAN 的敏感性和特异性。然后,排除 24 小时尿总蛋白<0.5 g/d、临床资料不全或随访时间<6 个月的患者,纳入 184 例 Non-MCD-IgAN 和 98 例 MCD-IgAN 患者。收集并比较患者的临床病理和结局数据。在 589 例患者中,根据电镜检查结果,381 例诊断为 Non-MCD-IgAN,167 例诊断为 MCD-IgAN,41 例诊断为合并其他肾小球疾病的 M-IgAN。以 NS 为诊断标准鉴别 Non-MCD-IgAN 和 MCD-IgAN,其敏感性和特异性分别为 83.8%和 99.5%。MCD-IgAN 组患者年龄较小、血压较低、尿红细胞较多,更易达到完全缓解,终点事件发生率较 Non-MCD-IgAN 组患者低(均 P<0.05)。NS 似乎是鉴别 MCD-IgAN 和 Non-MCD-IgAN 的客观指标。Non-MCD-IgAN 在临床病理和治疗反应方面与 MCD-IgAN 差异较大,预后较差。

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本文引用的文献

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Adult-onset minimal change disease: the significance of histological chronic changes for clinical presentation and outcome.成人起病型微小病变性肾病:组织学慢性改变对临床表现和结局的意义。
Clin Exp Nephrol. 2021 Mar;25(3):240-250. doi: 10.1007/s10157-020-01985-7. Epub 2020 Oct 22.
2
Consensus definitions for glomerular lesions by light and electron microscopy: recommendations from a working group of the Renal Pathology Society.肾小球病变的光镜和电镜共识定义:来自肾脏病理学会工作组的建议
Kidney Int. 2020 Nov;98(5):1120-1134. doi: 10.1016/j.kint.2020.08.006. Epub 2020 Aug 29.
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Oxford Classification of IgA nephropathy 2016: an update from the IgA Nephropathy Classification Working Group.
牛津 IgA 肾病分类 2016 年更新:IgA 肾病分类工作组的报告。
Kidney Int. 2017 May;91(5):1014-1021. doi: 10.1016/j.kint.2017.02.003. Epub 2017 Mar 22.
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IgA Nephropathy.IgA肾病
Clin J Am Soc Nephrol. 2017 Apr 3;12(4):677-686. doi: 10.2215/CJN.07420716. Epub 2017 Feb 3.
5
The relatively poor correlation between random and 24-hour urine protein excretion in patients with biopsy-proven glomerular diseases.经活检证实的肾小球疾病患者随机尿与24小时尿蛋白排泄之间的相关性相对较差。
Kidney Int. 2016 Nov;90(5):1080-1089. doi: 10.1016/j.kint.2016.06.020. Epub 2016 Aug 12.
6
Comparison between patients with IgA nephropathy with minimal change disease and patients with minimal change disease.IgA肾病合并微小病变病患者与微小病变病患者之间的比较。
Clin Nephrol. 2016 May;85(5):273-81. doi: 10.5414/CN108727.
7
Lack of electron microscopy hinders correct renal biopsy diagnosis: A study from India.缺乏电子显微镜检查妨碍肾脏活检的准确诊断:一项来自印度的研究。
Ultrastruct Pathol. 2016;40(1):14-7. doi: 10.3109/01913123.2015.1120837.
8
Long-term outcome of IgA nephropathy with minimal change disease: a comparison between patients with and without minimal change disease.IgA肾病合并微小病变的长期预后:有微小病变与无微小病变患者的比较。
J Nephrol. 2016 Aug;29(4):567-73. doi: 10.1007/s40620-015-0242-9. Epub 2015 Nov 4.
9
New developments in the genetics, pathogenesis, and therapy of IgA nephropathy.IgA肾病的遗传学、发病机制及治疗方面的新进展。
Kidney Int. 2015 Nov;88(5):974-89. doi: 10.1038/ki.2015.252. Epub 2015 Sep 16.
10
IgA nephropathy with minimal change disease.IgA 肾病伴微小病变病。
Clin J Am Soc Nephrol. 2014 Jun 6;9(6):1033-9. doi: 10.2215/CJN.11951113. Epub 2014 Apr 10.