Korean J Med.  2012 Oct;83(4):514-519.

Two Cases of Anti-Glomerular Basement Membrane Antibody Disease Associated with Perinuclear-Anti-Neutrophil Cytoplasmic Antibody

• Kang GW
• Hwang EA
• Han SY
• Park SB
• Kim HC
• Choe MS

Abstract

Anti-glomerular basement membrane antibody (anti-GBM Ab) disease is characterized by circulating antibodies to the glomerular basement membrane and the deposition of IgG or, rarely, IgA along the glomerular basement membrane. This disease accounts for 10-20% of crescentic glomerulonephritis. We report two patients with anti-GBM Ab disease who were positive for perinuclear-anti-neutrophil cytoplasmic antibody (p-ANCA). Percutaneous renal biopsies showed many crescent formations and linear deposits of IgG along the glomerular basement membrane. Serologic tests for p-ANCA were positive. They were treated with steroid pulse and cyclophosphamide and one patient also underwent plasma exchange therapy. Despite immunosuppressive therapy, their renal functions did not improve and both required regular hemodialysis.

Keyword

Anti-glomerular basement membrane antibody disease; Glomerulonephritis; Perinuclear-anti-neutrophil cytoplasmic antibody

MeSH Terms

Antibodies
Antibodies, Antineutrophil Cytoplasmic
Autoantibodies
Basement Membrane
Biopsy
Cyclophosphamide
Cytoplasm
Glomerular Basement Membrane
Glomerulonephritis
Hemorrhage
Humans
Immunoglobulin A
Immunoglobulin G
Lung Diseases
Plasma Exchange
Renal Dialysis
Serologic Tests
Antibodies
Antibodies, Antineutrophil Cytoplasmic
Autoantibodies
Cyclophosphamide
Glomerulonephritis
Hemorrhage
Immunoglobulin A
Immunoglobulin G
Lung Diseases