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利妥昔单抗治疗后出现DNAJB9相关性纤维性肾小球肾炎:一例暂时肾功能恢复的病例

DNAJB9 Fibrillary Glomerulonephritis Following Rituximab-Based Therapy: A Case of Temporary Renal Recovery.

作者信息

Ouanjine Arij, Fendri Fatma, Miquelestorena-Standley Elodie, Kerdraon Remy, Dekeyser Manon

机构信息

Department of Nephrology, Centre Hospitalier Universitaire d'Orléans, Orleans, FRA.

Department of Pathology, Centre Hospitalier Universitaire de Tours, Tours, FRA.

出版信息

Cureus. 2025 Jun 12;17(6):e85841. doi: 10.7759/cureus.85841. eCollection 2025 Jun.

DOI:10.7759/cureus.85841
PMID:40656342
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12255153/
Abstract

A 63-year-old man, with no relevant history, developed acute kidney injury with an elevated serum creatinine level of 314 µmol/L associated with hypertension, a nephrotic syndrome, without hematuria. Kidney biopsy revealed a glomerular-specific deposition of DnaJ homolog subfamily B member 9 (DNAJB9). Fibrillary glomerulonephritis was diagnosed. The patient received corticosteroids, rituximab (1 g at day 1 and day 15; 1 g at month 6), and nephroprotection. Kidney dysfunction initially worsened (creatinine 513 µmol/L), and peritoneal dialysis was initiated. Partial renal function recovery was observed after the rituximab-maintenance dose, allowing dialysis discontinuation for one year (October 2023 to September 2024). In some studies, rituximab-based therapy was associated with stabilization of disease progression. As observed in our case, it could be considered on a case-by-case basis, with a possible benefit for partial treatment response and time-limited disease control.

摘要

一名63岁男性,无相关病史,出现急性肾损伤,血清肌酐水平升高至314µmol/L,伴有高血压、肾病综合征,无血尿。肾活检显示DnaJ同源亚家族B成员9(DNAJB9)的肾小球特异性沉积。诊断为纤维样肾小球肾炎。患者接受了皮质类固醇、利妥昔单抗(第1天和第15天各1g;第6个月1g)以及肾脏保护治疗。肾功能最初恶化(肌酐513µmol/L),并开始进行腹膜透析。在利妥昔单抗维持剂量治疗后观察到部分肾功能恢复,使得透析中断了一年(2023年10月至2024年9月)。在一些研究中,基于利妥昔单抗的治疗与疾病进展稳定相关。如我们病例中所见,可根据具体情况考虑使用,可能对部分治疗反应和限时疾病控制有益。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6a59/12255153/aed4a9ec01e8/cureus-0017-00000085841-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6a59/12255153/01075e006a91/cureus-0017-00000085841-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6a59/12255153/dcf2731a9169/cureus-0017-00000085841-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6a59/12255153/aed4a9ec01e8/cureus-0017-00000085841-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6a59/12255153/01075e006a91/cureus-0017-00000085841-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6a59/12255153/dcf2731a9169/cureus-0017-00000085841-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6a59/12255153/aed4a9ec01e8/cureus-0017-00000085841-i03.jpg

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本文引用的文献

1
[Fibrillary glomerulonephritis].[纤维性肾小球肾炎]
Rev Med Interne. 2024 Nov;45(11):703-709. doi: 10.1016/j.revmed.2024.05.005. Epub 2024 May 15.
2
DNAJB9 Fibrillary Glomerulonephritis With Membranous-Like Pattern: A Case-Based Literature Review.具有膜样表现的DNAJB9相关性纤维性肾小球肾炎:基于病例的文献综述
Cureus. 2023 Oct 28;15(10):e47862. doi: 10.7759/cureus.47862. eCollection 2023 Oct.
3
Fibrillary Glomerulonephritis, DNAJB9, and the Unfolded Protein Response.纤维性肾小球肾炎、DNAJB9与未折叠蛋白反应
Glomerular Dis. 2022 Jun 16;2(4):164-175. doi: 10.1159/000525542. eCollection 2022.
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[Rituximab immunomonitoring in autoimmune diseases: a support tool in clinical practice?].[利妥昔单抗在自身免疫性疾病中的免疫监测:临床实践中的辅助工具?]
Rev Med Interne. 2021 Jun;42(6):384-391. doi: 10.1016/j.revmed.2021.02.006. Epub 2021 Mar 5.
5
DNAJB9-positive monotypic fibrillary glomerulonephritis is not associated with monoclonal gammopathy in the vast majority of patients.在绝大多数患者中,DNAJB9阳性单型纤维性肾小球肾炎与单克隆丙种球蛋白病无关。
Kidney Int. 2020 Aug;98(2):498-504. doi: 10.1016/j.kint.2020.02.025. Epub 2020 Mar 28.
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Treatment of fibrillary glomerulonephritis with rituximab: a 12-month pilot study.利妥昔单抗治疗纤维状肾小球肾炎:一项为期 12 个月的初步研究。
Nephrol Dial Transplant. 2021 Jan 1;36(1):104-110. doi: 10.1093/ndt/gfaa065.
7
Rituximab or Cyclosporine in the Treatment of Membranous Nephropathy.利妥昔单抗或环孢素治疗膜性肾病。
N Engl J Med. 2019 Jul 4;381(1):36-46. doi: 10.1056/NEJMoa1814427.
8
DNAJB9 Is a Specific Immunohistochemical Marker for Fibrillary Glomerulonephritis.DNAJB9是纤维性肾小球肾炎的一种特异性免疫组织化学标志物。
Kidney Int Rep. 2017 Aug 8;3(1):56-64. doi: 10.1016/j.ekir.2017.07.017. eCollection 2018 Jan.
9
DnaJ Homolog Subfamily B Member 9 Is a Putative Autoantigen in Fibrillary GN.DNAJ 同源物亚家族 B 成员 9 是纤维状肾小球肾炎的潜在自身抗原。
J Am Soc Nephrol. 2018 Jan;29(1):231-239. doi: 10.1681/ASN.2017050566. Epub 2017 Nov 2.
10
Rituximab treatment for fibrillary glomerulonephritis.利妥昔单抗治疗纤维样肾小球肾炎。
Nephrol Dial Transplant. 2014 Oct;29(10):1925-31. doi: 10.1093/ndt/gfu189. Epub 2014 May 27.