Korean J Med.  2012 Dec;83(6):807-812.

A Case of P-ANCA-Positive Anti-Glomerular Basement Membrane Antibody Disease

• Chung HA
• Son IS
• Hwang Y
• Choi HS
• Kim DY
• Lim SD
• Jo YI

Abstract

Up to 40% of patients with anti-glomerular basement membrane (GBM) disease, which is a rare autoimmune disorder usually manifesting as rapidly progressive glomerulonephritis (RPGN), are positive for circulating anti-neutrophil cytoplasmic antibody (ANCA). Many previous reports showed poor outcomes in these "double-positive" patients. We report a patient with perinuclear (p)-ANCA positive anti-GBM disease who presented with RPGN and required hemodialysis. Plasmapheresis and steroid and cyclophosphamide therapy were initiated following renal biopsy and resulted in normalization of anti-GBM antibody and p-ANCA titers, recovery of renal function, and discontinuation of hemodialysis. This case suggests that aggressive immunosuppression with plasmapheresis in patients who are p-ANCA positive with anti-GBM disease should be considered, even in those with severe renal dysfunction.

Keyword

Anti-glomerular basement membrane antibody disease; ANCA; Glomerulonephritis

MeSH Terms

Anti-Glomerular Basement Membrane Disease
Antibodies, Antineutrophil Cytoplasmic
Autoantibodies
Basement Membrane
Biopsy
Cyclophosphamide
Glomerulonephritis
Hemorrhage
Humans
Immunosuppression
Lung Diseases
Plasmapheresis
Renal Dialysis
Antibodies, Antineutrophil Cytoplasmic
Autoantibodies
Cyclophosphamide
Glomerulonephritis
Hemorrhage
Lung Diseases