A Rare Case of Atypical Anti-Glomerular Basement Membrane Disease Combined with Diabetic Nephropathy and Literature Review

Introduction: The atypical anti-GBM disease is a rare, diagnostically challenging, and rapidly progressive condition. Case Description: We report a case of a 59-year-old male who exhibited an insidious onset of hematuria, nephrotic syndrome, and mild renal dysfunction, with negative serum anti-glomerular basement membrane antibodies. The initial renal biopsy diagnosed diabetic nephropathy. As the disease progressed, the clinical manifestations evolved into rapidly progressive glomerulonephritis, and a second kidney biopsy ultimately diagnosed atypical anti-glomerular basement membrane disease in conjunction with diabetic nephropathy. Furthermore, we incubated normal renal tissue with both the patient's serum and serum from an anti-GBM antibody-positive patient, and observed identical linear IgG deposition along the basement membrane. This result indirectly suggests the presence of anti-GBM antibodies in the patient's serum. Despite receiving immunosuppressive treatment, renal function did not improve, and the patient ultimately required maintenance renal replacement therapy. Discussion: This case highlights the importance of closely monitoring disease progression in atypical presentations of rare diseases and emphasizes the necessity of repeat renal biopsies for the accurate diagnosis of atypical anti-glomerular basement membrane disease. We diagnosed the anti-GBM disease using an indirect immunofluorescence antibody method and we need more detection methods to determine the target antigen.

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Publication Details

Journal
Journal of the American Society of Nephrology
Published
2025-10-01
DOI
https://doi.org/10.1681/asn.2025nppr2w0z
Primary Topic
Vasculitis and related conditions
Type
article
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article

A Rare Case of Atypical Anti-Glomerular Basement Membrane Disease Combined with Diabetic Nephropathy and Literature Review

Journal of the American Society of Nephrology
Vasculitis and related conditions
article

A Rare Case of Atypical Anti-Glomerular Basement Membrane Disease Combined with Diabetic Nephropathy and Literature Review

article en

Abstract

Introduction: The atypical anti-GBM disease is a rare, diagnostically challenging, and rapidly progressive condition. Case Description: We report a case of a 59-year-old male who exhibited an insidious onset of hematuria, nephrotic syndrome, and mild renal dysfunction, with negative serum anti-glomerular basement membrane antibodies. The initial renal biopsy diagnosed diabetic nephropathy. As the disease progressed, the clinical manifestations evolved into rapidly progressive glomerulonephritis, and a second kidney biopsy ultimately diagnosed atypical anti-glomerular basement membrane disease in conjunction with diabetic nephropathy. Furthermore, we incubated normal renal tissue with both the patient's serum and serum from an anti-GBM antibody-positive patient, and observed identical linear IgG deposition along the basement membrane. This result indirectly suggests the presence of anti-GBM antibodies in the patient's serum. Despite receiving immunosuppressive treatment, renal function did not improve, and the patient ultimately required maintenance renal replacement therapy. Discussion: This case highlights the importance of closely monitoring disease progression in atypical presentations of rare diseases and emphasizes the necessity of repeat renal biopsies for the accurate diagnosis of atypical anti-glomerular basement membrane disease. We diagnosed the anti-GBM disease using an indirect immunofluorescence antibody method and we need more detection methods to determine the target antigen.

Journal of the American Society of NephrologyVol. 36(10S)
Beijing Anzhen Hospital (CN)
Good health and well-being
Openalex Percentile: Top 32%
Vasculitis and related conditions
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