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Membranous Nephropathy with Proteinase 3-ANCA-associated Vasculitis Successfully Treated with Rituximab

Shun Yoshida1, Shunichiro Hanai1, Daiki Nakagomi1

  • 1Third Department of Internal Medicine, University of Yamanashi, Japan.

Internal Medicine (Tokyo, Japan)
|August 25, 2020
PubMed

Insights

Membranous nephropathy (MN) can occur with anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis (ANCA-GN). This case highlights proteinase 3 (PR3)-ANCA

Area of Science:

  • Nephrology
  • Immunology
  • Pathology

Background:

  • Membranous nephropathy (MN) is a kidney disease characterized by immune complex deposition in the glomerular basement membrane.
  • Anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis (ANCA-GN) is typically associated with different autoantibodies.
  • The co-occurrence of MN and ANCA-GN is infrequent, with myeloperoxidase-ANCA (MPO-ANCA) being the most commonly reported ANCA subtype in such cases.

Observation:

  • A 73-year-old woman presented with scleritis, hematuria, and proteinuria.
  • Serum testing revealed positive proteinase 3 (PR3)-ANCA.
  • Renal biopsy confirmed a diagnosis of both MN and ANCA-GN.

Findings:

  • Immunofluorescence microscopy demonstrated co-localization of PR3 with IgG along the glomerular basement membrane.
  • This finding suggests a potential role for PR3 in the immune complex formation characteristic of MN.
  • Treatment with oral prednisolone and intravenous rituximab led to rapid clinical improvement and resolution of urinalysis abnormalities.

Implications:

  • Proteinase 3 (PR3)-ANCA may play a direct role in the pathogenesis of membranous nephropathy.
  • This case expands the understanding of ANCA-GN subtypes and their association with glomerular diseases.
  • The findings suggest that PR3-ANCA should be considered in the differential diagnosis of MN, particularly in patients with systemic inflammatory symptoms.
Keywords:
glomerular diseaseimmune complex depositsimmunofluorescence stainingsecondary membranous nephropathysmall vessel vasculitis

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