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Abstract: TH-PO974

A Case of Anti-Glomerular Basement Membrane Disease Recognized a Month After the Diagnosis of Membranous Nephropathy

Session Information

Category: Trainee Case Report

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Kawaji, Atsuro, Division of Nephrology, Endocrinology and Metabolism, Tokai University School of Medicine, Isehara, Japan
  • Nakagawa, Yosuke, Division of Nephrology, Endocrinology and Metabolism, Tokai University School of Medicine, Isehara, Japan
  • Hamano, Naoto, Division of Nephrology, Endocrinology and Metabolism, Tokai University School of Medicine, Isehara, Japan
  • Koizumi, Masahiro, Division of Nephrology, Endocrinology and Metabolism, Tokai University School of Medicine, Isehara, Japan
  • Wada, Takehiko, Division of Nephrology, Endocrinology and Metabolism, Tokai University School of Medicine, Isehara, Japan
  • Fukagawa, Masafumi, Division of Nephrology, Endocrinology and Metabolism, Tokai University School of Medicine, Isehara, Japan
Introduction

Most of the patients with anti-glomerular basement membrane (GBM) disease present with rapidly progressive glomerulonephritis. However, in rare cases, this disorder develops following membranous nephropathy (MN). Here, we report a case of anti-GBM disease which developed shortly after the diagnosis of MN.

Case Description

A 76-year-old Japanese woman with hypertension and dyslipidemia was found to have nephrotic-range proteinuria and microscopic hematuria without increase in serum creatinine level. Although a renin-angiotensin system inhibitor was initiated, massive proteinuria persisted and hypoalbuminemia had deteriorated over the following ten months, resulting in nephrotic syndrome. Kidney biopsy demonstrated thickening of GBM with a diffuse granular pattern of IgG and C3 staining along GBM on immunofluorescence microscopy. Electron microscopy revealed subepithelial and intramembranous electron-dense deposits, which led to the diagnosis of MN (stage II-III). At this diagnosis of MN, anti-GBM antibody was not detected in her serum. One month later, she was admitted with persistent fever and oliguria. She got anuric soon after the admission and hemodialysis (HD) therapy was initiated. Laboratory test revealed a high titer of anti-GBM antibody (276 IU/mL), and plasmapheresis therapy combined with corticosteroids was also initiated. The second biopsy showed severe necrotizing glomerulonephritis throughout all glomeruli with cellular crescents. Immunofluorescence microscopy demonstrated deposition of IgG along GBM in both linear and granular patterns. Her renal function did not recover in spite of decrease in titer of anti-GBM antibody, and she has been eventually on maintenance HD.

Discussion

The conformational change of GBM by MN may unmask hidden epitope in the non-collagenous domain 1 of typeIVcollagen, which could induce autoimmune response. Further studies are needed to determine whether MN could cause such autoimmunity.