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伴膜性增生的非典型抗肾小球基底膜病:诊断挑战和治疗变异性。

Atypical anti-glomerular basement membrane disease with membranous hyperplasia: diagnostic challenges and treatment variability.

机构信息

Nephrology Department, Chinese PLA Southern Theater Command General Hospital, 510010, Guangzhou, China.

Pathology Department, Chinese PLA Southern Theater Command General Hospital, 510010, Guangzhou, China.

出版信息

BMC Nephrol. 2024 Apr 15;25(1):132. doi: 10.1186/s12882-024-03571-5.

Abstract

This case report presents a detailed analysis of a 31-year-old male patient who presented with a complex array of clinical symptoms, including proteinuria, hematuria, edema, and kidney insufficiency. Despite undergoing multiple tests, the results for anti-glomerular basement membrane antibodies yielded negative findings. Subsequently, kidney biopsy pathology revealed a distinct diagnosis of atypical anti-glomerular basement membrane (anti-GBM) disease with membrane hyperplasia. Treatment was initiated with a comprehensive approach involving high doses of corticosteroids therapy and cyclophosphamide (CTX). However, contrary to expectations, the patient's kidney function exhibited rapid deterioration following this therapeutic regimen. The culmination of these complications necessitated a pivotal transition to maintenance hemodialysis. This case underscores the intricate challenges associated with diagnosing and managing rare and atypical presentations of kidney disorders. The negative anti-GBM antibody results and subsequent identification of atypical anti-GBM nephropathy highlight the need for tailored diagnostic strategies to discern subtle nuances within complex clinical scenarios. Additionally, the unexpected response to the treatment regimen emphasizes the potential variability in individual patient responses, underlining the necessity for vigilant monitoring and adaptable treatment strategies. This case report contributes to the evolving understanding of atypical kidney pathologies and the complexities involved in their management.

摘要

本病例报告详细分析了一位 31 岁男性患者,他表现出一系列复杂的临床症状,包括蛋白尿、血尿、水肿和肾功能不全。尽管进行了多项检查,但抗肾小球基底膜抗体的结果呈阴性。随后,肾脏活检病理显示明确的非典型抗肾小球基底膜(anti-GBM)疾病伴膜增生。治疗采用包括大剂量皮质类固醇和环磷酰胺(CTX)在内的综合方法开始。然而,与预期相反,在这种治疗方案后,患者的肾功能迅速恶化。这些并发症的综合作用需要进行关键的转变,以维持血液透析。本病例突出了诊断和管理罕见和非典型肾脏疾病表现所涉及的复杂挑战。抗 GBM 抗体阴性结果和随后鉴定出的非典型抗 GBM 肾病强调需要制定针对性的诊断策略,以辨别复杂临床情况下的细微差异。此外,对治疗方案的意外反应强调了个体患者反应的潜在可变性,突出了需要进行警惕监测和适应性治疗策略的必要性。本病例报告有助于加深对非典型肾脏病理的理解以及其管理所涉及的复杂性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e39a/11017607/f6159049a0c7/12882_2024_3571_Fig1_HTML.jpg

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