TY - JOUR
T1 - Rapidly progressive glomerulonephritis due to coexistent anti-glomerular basement membrane disease and fibrillary glomerulonephritis
AU - Cheungpasitporn, Wisit
AU - Zacharek, Claudia C.
AU - Fervenza, Fernando C.
AU - Cornell, Lynn D.
AU - Sethi, Sanjeev
AU - Herrera Hernandez, Loren P.
AU - Nasr, Samih H.
AU - Alexander, Mariam P.
N1 - Publisher Copyright:
© The Author 2015.
PY - 2016/2/1
Y1 - 2016/2/1
N2 - Anti-glomerular basement membrane (anti-GBM) disease is a major cause of rapidly progressive glomerulonephritis (RPGN). On the other hand, fibrillary glomerulonephritis (GN) typically presents as proteinuria, hematuria and renal insufficiency, but rarely as RPGN. Without electron microscopy, the diagnosis of fibrillary GN can be missed.We report a 68-year-old white woman who presented with RPGN with kidney biopsy demonstrating diffuse crescentic GN on light microscopy. By immunofluorescence, there was bright linear staining of the GBMs and smudgy mesangial staining for immunoglobulin G, C3, and kappa and lambda light chain. Electron microscopy revealed fibrillary deposits in the GBM and mesangium. A serum test for anti-GBM antibody was positive. To our knowledge, this is the first report of coexistence of fibrillary GN in a patient with anti-GBM disease. Electron microscopy is critical to identify the coexistence of other GN in patients presenting with crescentic GN.
AB - Anti-glomerular basement membrane (anti-GBM) disease is a major cause of rapidly progressive glomerulonephritis (RPGN). On the other hand, fibrillary glomerulonephritis (GN) typically presents as proteinuria, hematuria and renal insufficiency, but rarely as RPGN. Without electron microscopy, the diagnosis of fibrillary GN can be missed.We report a 68-year-old white woman who presented with RPGN with kidney biopsy demonstrating diffuse crescentic GN on light microscopy. By immunofluorescence, there was bright linear staining of the GBMs and smudgy mesangial staining for immunoglobulin G, C3, and kappa and lambda light chain. Electron microscopy revealed fibrillary deposits in the GBM and mesangium. A serum test for anti-GBM antibody was positive. To our knowledge, this is the first report of coexistence of fibrillary GN in a patient with anti-GBM disease. Electron microscopy is critical to identify the coexistence of other GN in patients presenting with crescentic GN.
KW - anti-GBM
KW - anti-glomerular basementmembrane disease
KW - crescentic glomerulonephritis
KW - fibrillary glomerulonephritis
KW - rapidly progressive glomerulonephritis
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U2 - 10.1093/ckj/sfv126
DO - 10.1093/ckj/sfv126
M3 - Article
AN - SCOPUS:85007233550
SN - 2048-8505
VL - 9
SP - 97
EP - 101
JO - CKJ: Clinical Kidney Journal
JF - CKJ: Clinical Kidney Journal
IS - 1
ER -