A Case of Concurrent MPO-/PR3-Negative ANCA-Associated Glomerulonephritis and Membranous Glomerulopathy

Yasuyuki Nakada1, Nobuo Tsuboi1, Yasuto Takahashi1

  • 1Division of Nephrology and Hypertension, Department of Internal Medicine, The Jikei University School of Medicine, 3-25-8 Nishi-Shinbashi, Minato-ku, Tokyo 105-8461, Japan.

Insights

This case study details a patient with concurrent antineutrophil cytoplasmic antibody- (ANCA-) associated glomerulonephritis and membranous glomerulopathy (MGN). The ANCA was not MPO or PR3 but targeted other proteins, highlighting a complex renal presentation.

Area of Science:

  • Nephrology
  • Immunology
  • Pathology

Background:

  • Concurrent glomerulonephritis and membranous glomerulopathy are rare.
  • Antineutrophil cytoplasmic antibody- (ANCA-) associated glomerulonephritis typically targets myeloperoxidase (MPO) or proteinase 3 (PR3).

Purpose of the Study:

  • To report a unique case of co-existing ANCA-associated glomerulonephritis and membranous glomerulopathy.
  • To characterize the specific ANCA targets in a patient with rapidly progressive renal failure.

Main Methods:

  • Renal biopsy analysis for glomerulonephritis and membranous glomerulopathy.
  • Indirect immunofluorescence for ANCA pattern.
  • Enzyme-linked immunosorbent assays (ELISAs) to identify ANCA targets.

Main Results:

  • The patient presented with rapidly progressive renal deterioration.
  • Renal biopsy revealed crescentic glomerulonephritis and membranous glomerulopathy with characteristic GBM changes.
  • ANCA testing showed a perinuclear pattern but did not target MPO or PR3.
  • ELISAs identified ANCA targeting bactericidal-/permeability-increasing protein, elastase, and lysosome.

Conclusions:

  • This case demonstrates MPO-/PR3-negative ANCA-associated glomerulonephritis occurring concurrently with membranous glomerulopathy.
  • The pathogenic relationship between these two distinct renal diseases in this patient remains to be elucidated.