A Case of P-ANCA-Positive Anti-Glomerular Basement Membrane Antibody Disease.
- Author:
Hyun Ah CHUNG
1
;
In Sung SON
;
Yong HWANG
;
Hong Seok CHOI
;
Do Young KIM
;
So Duk LIM
;
Young Il JO
Author Information
1. Department of Internal Medicine, Konkuk University School of Medicine, Seoul, Korea. nephjo@kuh.ac.kr
- Publication Type:Case Report
- Keywords:
Anti-glomerular basement membrane antibody disease;
ANCA;
Glomerulonephritis
- MeSH:
Anti-Glomerular Basement Membrane Disease;
Antibodies, Antineutrophil Cytoplasmic;
Autoantibodies;
Basement Membrane;
Biopsy;
Cyclophosphamide;
Glomerulonephritis;
Hemorrhage;
Humans;
Immunosuppression;
Lung Diseases;
Plasmapheresis;
Renal Dialysis
- From:Korean Journal of Medicine
2012;83(6):807-812
- CountryRepublic of Korea
- Language:Korean
-
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.
