A Case of P-ANCA-Positive Anti-Glomerular Basement Membrane Antibody Disease / 대한내과학회지
Korean Journal of Medicine
;
: 807-812, 2012.
Article
in Korean
| WPRIM
| ID: wpr-126592
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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Autoantibodies
/
Basement Membrane
/
Biopsy
/
Renal Dialysis
/
Immunosuppression Therapy
/
Plasmapheresis
/
Antibodies, Antineutrophil Cytoplasmic
/
Anti-Glomerular Basement Membrane Disease
/
Cyclophosphamide
/
Glomerulonephritis
Limits:
Humans
Language:
Korean
Journal:
Korean Journal of Medicine
Year:
2012
Type:
Article
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