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伴单克隆免疫球蛋白沉积和非典型病理表现的增生性肾小球肾炎,采用皮质类固醇和利妥昔单抗治疗。

Proliferative glomerulonephritis with monoclonal immunoglobulin deposits and atypical pathological findings treated with corticosteroid and rituximab.

机构信息

Department of Nephrology, Nagoya University Hospital, Nagoya, Aichi, Japan.

Department of Nephrology, Nagoya University Graduate School of Medicine, Nagoya, Aichi, Japan.

出版信息

CEN Case Rep. 2024 Apr;13(2):128-134. doi: 10.1007/s13730-023-00813-7. Epub 2023 Aug 7.

DOI:10.1007/s13730-023-00813-7
PMID:37548878
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10982278/
Abstract

A 16-year-old girl with fever that appeared after taking the second COVID-19 vaccine presented to the clinic with a serum creatinine of 0.89 mg/dL and C-reactive protein of 6.9 mg/dL. She had proteinuria and microscopic hematuria, with slowly worsening kidney function. Her kidney biopsy showed fibrocellular crescents in seven of nine glomeruli that were observed under light microscopy. Another glomerulus showed endocapillary hypercellularity and mesangial cell proliferation. Electron-dense deposits were significant in the mesangial area, with monoclonal IgG1-κ and C3 deposition by immunofluorescence. The patient was diagnosed with proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) and atypical pathological finding of diffuse crescent formation. The treatment regimen for PGNMID has not yet been established, and the appropriate duration of treatment is unknown. In our case, considering that rituximab acts by binding to CD20 on the surface of B cells through its crystallizable fragment, it was administered in addition to prednisolone, which successfully decreased the proteinuria over time.

摘要

一位 16 岁女孩在接种第二剂 COVID-19 疫苗后出现发热,因血清肌酐 0.89mg/dL 和 C 反应蛋白 6.9mg/dL 就诊。她有蛋白尿和镜下血尿,肾功能逐渐恶化。肾脏活检显示在 9 个肾小球中 7 个肾小球有纤维细胞性新月体,在光镜下观察到。另一个肾小球显示毛细血管内细胞增生和系膜细胞增殖。电子致密沉积物在系膜区明显,免疫荧光显示单克隆 IgG1-κ 和 C3 沉积。该患者被诊断为增生性肾小球肾炎伴单克隆免疫球蛋白沉积(PGNMID)和弥漫性新月体形成的非典型病理发现。PGNMID 的治疗方案尚未确定,治疗的适当持续时间尚不清楚。在我们的病例中,由于利妥昔单抗通过其可结晶片段与 B 细胞表面的 CD20 结合而发挥作用,因此在泼尼松龙的基础上加用利妥昔单抗,随着时间的推移成功地减少了蛋白尿。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5681/10982278/c201e3bda262/13730_2023_813_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5681/10982278/7aba4fc22882/13730_2023_813_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5681/10982278/c201e3bda262/13730_2023_813_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5681/10982278/7aba4fc22882/13730_2023_813_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5681/10982278/c201e3bda262/13730_2023_813_Fig2_HTML.jpg

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