伴膜增生病变的非典型抗肾小球基底膜肾炎1例报道

doi:10.3969/j.issn.1674-8115.2024.11.016

上海交通大学学报（医学版） ›› 2024, Vol. 44 ›› Issue (11): 1472-1476.doi: 10.3969/j.issn.1674-8115.2024.11.016

• 病例报告 • 上一篇

伴膜增生病变的非典型抗肾小球基底膜肾炎1例报道

张昕萍¹(), 王自闯², 陈小永¹()

^1.河南中医药大学第二临床医学院肾病科，郑州 450003
^2.河南中医药大学第二临床医学院病理科，郑州 450003

收稿日期:2024-01-24 接受日期:2024-06-24 出版日期:2024-11-28 发布日期:2024-11-28
通讯作者: 陈小永 E-mail:2783276385@qq.com;chenxy603@sina.com
作者简介:张昕萍（1999—），女，住院医师，硕士生；电子信箱：2783276385@qq.com。

A case report of atypical anti-glomerular basement membrane disease with membrane hyperplasia lesions

ZHANG Xinping¹(), WANG Zichuang², CHEN Xiaoyong¹()

^1.Department of Nephrology, Second Clinical Medical College, Henan University of Chinese Medicine, Zhengzhou 450003, China
^2.Department of Pathology, Second Clinical Medical College, Henan University of Chinese Medicine, Zhengzhou 450003, China

Received:2024-01-24 Accepted:2024-06-24 Online:2024-11-28 Published:2024-11-28
Contact: CHEN Xiaoyong E-mail:2783276385@qq.com;chenxy603@sina.com

摘要/Abstract

摘要：

非典型抗肾小球基底膜（glomerular basement membrane，GBM）肾炎罕见，伴有膜增生病变的更为少见，目前对其治疗方案尚未明确。该文报道1例抗GBM抗体阴性，但肾组织活检提示“肾小球膜增生病变伴IgG线样阳性”的非典型抗GBM肾炎病例。该患者以大量蛋白尿、血尿、水肿、肾功能损伤为主要特征，排除继发因素后经肾组织活检诊断为“非典型抗GBM肾炎”。给予糖皮质激素联合环磷酰胺治疗后，患者出现肺部感染及急性左心衰竭，转院行规律血液透析治疗。后期发展至终末期肾脏病阶段，继续接受规律血液透析治疗。

关键词: 非典型抗肾小球基底膜肾炎, 膜增生性病变, 肾病综合征

Abstract:

Atypical anti-glomerular basement membrane (GBM) disease is rare, and the atypical anti-GBM disease with membrane hyperplasia lesion is even rarer. The treatment plan for it is not clear. This article reports a case of atypical anti-GBM disease with a negative anti-GBM antibody test, but renal tissue biopsy showed “glomerular membrane hyperplasia lesions with positive IgG linearity”, which provides a reference for clinical diagnosis and treatment. The patient exhibited massive proteinuria, hematuria, edema, and renal impairment. After ruling out secondary factors, the patient was diagnosed with “atypical anti-GBM nephritis” by renal tissue biopsy, and was treated with glucocorticoids combined with cyclophosphamide, after which the patient developed a lung infection and acute left heart failure, and received regular hemodialysis treatment. Then the patient progressed to the stage of end-stage renal disease and continued to receive regular hemodialysis treatment.

Key words: atypical anti-glomerular basement membrane nephritis, membrane proliferative lesion, nephrotic syndrome

中图分类号:

692.3

张昕萍, 王自闯, 陈小永. 伴膜增生病变的非典型抗肾小球基底膜肾炎1例报道[J]. 上海交通大学学报（医学版）, 2024, 44(11): 1472-1476.

ZHANG Xinping, WANG Zichuang, CHEN Xiaoyong. A case report of atypical anti-glomerular basement membrane disease with membrane hyperplasia lesions[J]. Journal of Shanghai Jiao Tong University (Medical Science), 2024, 44(11): 1472-1476.

图/表 3

图1 光学显微镜下观察肾脏组织（×400）Note： A/B. Hematoxylin-eosin (H-E) staining (A) and periodic acid-silver methenamine (PASM) staining (B) show mesangial cells and stroma with moderate to severe hyperplasia, thickening of the basement membrane, segmental mesangial insertion, and “double track sign” formation. C. Periodic acid schiff (PAS) staining show microcytic crescent formation.

Fig 1 Observation of renal tissue under an optical microscope (×400)

图2 免疫荧光染色观察患者肾脏组织（×200）Note： A. IgG is deposited in a linear manner along the glomerular capillaries, and some distal tubular basement membranes are positive. B. Positive staining for IgG1. C/D. Positive light chain kappa (C) and lambda (D). E. C3 deposition along the mesangial region of the glomerulus in granular form.

Fig 2 Observation of renal tissue by immunofluorescence (×200)

图3 电子显微镜下观察肾脏组织超微结构Note： A. The glomerular basement membrane was segmentally thickened, and the foot process was diffusely fused. B. The mesangial cells and matrix proliferation were obvious, and a small amount of low-density electron dense matter was deposited in the mesangial area. C. Capillary endothelial cells vacuole degeneration, red blood cells visible in the lumen.

Fig 3 Observation of renal tissue ultrastructure under electron microscope

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伴膜增生病变的非典型抗肾小球基底膜肾炎1例报道

A case report of atypical anti-glomerular basement membrane disease with membrane hyperplasia lesions

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