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双抗体阳性急进性肾小球肾炎中的肾脏局限性抗肾小球基底膜病:一例报告

Renal-Limited Anti-GBM Disease in Dual-Antibody Positive RPGN: A Case Report.

作者信息

Guo Wilson, O'Donnell Michael

机构信息

Warren Alpert Medical School of Brown University.

Division of Hospital Medicine, Department of Medicine Brown University Health.

出版信息

J Brown Hosp Med. 2025 Jul 1;4(3):141754. doi: 10.56305/001c.141754. eCollection 2025.

DOI:10.56305/001c.141754
PMID:40641986
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12243613/
Abstract

Anti-glomerular basement membrane (anti-GBM) disease is a rare autoimmune disorder causing rapidly progressive glomerulonephritis. Early recognition is crucial to prevent irreversible damage. We present a 70-year-old female with hypertension and COPD who had elevated creatinine without respiratory symptoms. Workup revealed elevated anti-GBM antibodies (59 U/mL), anti-MPO antibodies (168 AU/mL), and p-ANCA positivity with kidney biopsy confirming crescentic glomerulonephritis. She was treated with plasmapheresis, corticosteroids, and cyclophosphamide. Her antibody titers declined, and she was discharged on hemodialysis and immunosuppressive therapy. At her 4-week follow-up, renal function had not recovered, and she continued to require hemodialysis. This case highlights the need to consider anti-GBM disease even in isolated renal presentations and emphasizes challenges in managing dual-positive cases. Long-term considerations include maintenance immunosuppression and infection prevention.

摘要

抗肾小球基底膜(anti-GBM)病是一种罕见的自身免疫性疾病,可导致快速进展性肾小球肾炎。早期识别对于预防不可逆损伤至关重要。我们报告一例70岁患有高血压和慢性阻塞性肺疾病(COPD)的女性,其肌酐升高但无呼吸道症状。检查发现抗GBM抗体升高(59 U/mL)、抗髓过氧化物酶(MPO)抗体升高(168 AU/mL),且p-ANCA呈阳性,肾活检证实为新月体性肾小球肾炎。她接受了血浆置换、皮质类固醇和环磷酰胺治疗。她的抗体滴度下降,出院时接受血液透析和免疫抑制治疗。在4周的随访中,肾功能未恢复,她仍继续需要血液透析。该病例强调即使在孤立的肾脏表现中也需要考虑抗GBM病,并强调了管理双阳性病例的挑战。长期考虑包括维持免疫抑制和预防感染。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4909/12243613/b6779b9e2f95/bhm_2025_4_3_141754_291854.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4909/12243613/14a2079c2bc5/bhm_2025_4_3_141754_291852.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4909/12243613/b6779b9e2f95/bhm_2025_4_3_141754_291854.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4909/12243613/14a2079c2bc5/bhm_2025_4_3_141754_291852.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4909/12243613/b6779b9e2f95/bhm_2025_4_3_141754_291854.jpg

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本文引用的文献

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Anti-Glomerular Basement Membrane Disease: Recent Updates.抗肾小球基底膜病:最新进展。
Adv Kidney Dis Health. 2024 May;31(3):206-215. doi: 10.1053/j.akdh.2024.04.007.
2
Renal and overall outcomes of double-positive (ANCA and anti-GBM antibodies) patients compared to ANCA-associated vasculitis patients with severe renal involvement: A multicenter retrospective study with systematic renal pathology analysis.与伴有严重肾脏受累的抗中性粒细胞胞浆抗体(ANCA)相关血管炎患者相比,双阳性(ANCA和抗肾小球基底膜抗体)患者的肾脏及总体预后:一项进行系统性肾脏病理分析的多中心回顾性研究。
Scand J Rheumatol. 2022 May;51(3):205-213. doi: 10.1080/03009742.2021.1920120. Epub 2021 Jun 25.
3
Rituximab in treatment of anti-GBM antibody glomerulonephritis: A case report and literature review.
利妥昔单抗治疗抗肾小球基底膜抗体肾小球肾炎:一例报告及文献综述
Medicine (Baltimore). 2019 Nov;98(44):e17801. doi: 10.1097/MD.0000000000017801.
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Anti-Glomerular Basement Membrane Disease.抗肾小球基底膜病
Clin J Am Soc Nephrol. 2017 Jul 7;12(7):1162-1172. doi: 10.2215/CJN.01380217. Epub 2017 May 17.
5
Patients double-seropositive for ANCA and anti-GBM antibodies have varied renal survival, frequency of relapse, and outcomes compared to single-seropositive patients.与单阳性患者相比,同时出现抗中性粒细胞胞浆抗体(ANCA)和抗肾小球基底膜(GBM)抗体双阳性的患者具有不同的肾脏存活率、复发频率和结局。
Kidney Int. 2017 Sep;92(3):693-702. doi: 10.1016/j.kint.2017.03.014. Epub 2017 May 12.
6
Rituximab in ANCA-Associated Vasculitis.利妥昔单抗治疗抗中性粒细胞胞浆抗体相关性血管炎
Curr Rheumatol Rep. 2017 Feb;19(2):6. doi: 10.1007/s11926-017-0632-1.
7
Clustering of Anti-GBM Disease: Clues to an Environmental Trigger?抗肾小球基底膜病的聚集现象:环境触发因素的线索?
Clin J Am Soc Nephrol. 2016 Aug 8;11(8):1324-1326. doi: 10.2215/CJN.05580516. Epub 2016 Jul 11.
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Predictors of renal and patient outcomes in anti-GBM disease: clinicopathologic analysis of a two-centre cohort.抗肾小球基底膜病中肾脏及患者预后的预测因素:一项两中心队列的临床病理分析
Nephrol Dial Transplant. 2015 May;30(5):814-21. doi: 10.1093/ndt/gfu399. Epub 2015 Jan 20.
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