Two Cases of Anti-Glomerular Basement Membrane Antibody Disease Associated with Perinuclear-Anti-Neutrophil Cytoplasmic Antibody.
- Author:
Gun Woo KANG
1
;
Eun Ah HWANG
;
Seung Yeup HAN
;
Sung Bae PARK
;
Hyun Chul KIM
;
Mi Sun CHOE
Author Information
1. Department of Internal Medicine, Keimyung University School of Medicine, Daegu, Korea. hyunkim48@gmail.com
- Publication Type:Case Report
- Keywords:
Anti-glomerular basement membrane antibody disease;
Glomerulonephritis;
Perinuclear-anti-neutrophil cytoplasmic antibody
- MeSH:
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
- From:Korean Journal of Medicine
2012;83(4):514-519
- CountryRepublic of Korea
- Language:Korean
-
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.