A case of ANCA-negative renal small-vessel vasculitis with tubulointerstitial infiltration of IgG4-positive plasma cells.
A 62-year-old male patient presented with progressive renal dysfunction for 2 months. He had elevated serum C-reactive protein and IgG4 levels with absence of anti-neutrophil cytoplasmic antibodies. A renal biopsy showed severe tubulointerstitial nephritis (TIN) with extensive infiltration of IgG4-positive plasma cells, suggesting a diagnosis of IgG4-related kidney disease (IgG4-RKD). However, the identification of a few crescentic glomeruli and necrotizing vasculitis of an interlobular artery lead to a diagnosis of renal small-vessel vasculitis. This case indicates that a careful examination is required to distinguish between IgG4-RKD and TIN caused by renal small-vessel vasculitis.
a Department of Medicine and Clinical Science , Gunma University Graduate School of Medicine , Maebashi , Gunma , Japan.
Toru
T
Sakairi
a Department of Medicine and Clinical Science , Gunma University Graduate School of Medicine , Maebashi , Gunma , Japan.
Satoshi
S
Okabe
a Department of Medicine and Clinical Science , Gunma University Graduate School of Medicine , Maebashi , Gunma , Japan.
Keiju
K
Hiromura
a Department of Medicine and Clinical Science , Gunma University Graduate School of Medicine , Maebashi , Gunma , Japan.
Shinsuke
S
Motegi
a Department of Medicine and Clinical Science , Gunma University Graduate School of Medicine , Maebashi , Gunma , Japan.
Noriyuki
N
Sakurai
a Department of Medicine and Clinical Science , Gunma University Graduate School of Medicine , Maebashi , Gunma , Japan.
Hidekazu
H
Ikeuchi
a Department of Medicine and Clinical Science , Gunma University Graduate School of Medicine , Maebashi , Gunma , Japan.
Yoriaki
Y
Kaneko
a Department of Medicine and Clinical Science , Gunma University Graduate School of Medicine , Maebashi , Gunma , Japan.
Akito
A
Maeshima
b Department of Pathology , Gunma University Hospital , Maebashi , Gunma , Japan.
Junko
J
Hirato
a Department of Medicine and Clinical Science , Gunma University Graduate School of Medicine , Maebashi , Gunma , Japan.
Yoshihisa
Y
Nojima
D019268:Antibodies, Antineutrophil Cytoplasmic; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*; D007668:Kidney / Q000276:immunology / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D010950:Plasma Cells / Q000276:immunology / Q000473:pathology*; D014657:Vasculitis / Q000276:immunology / Q000473:pathology*
D019268:Antibodies, Antineutrophil Cytoplasmic; D007074:Immunoglobulin G
ANCA-associated vasculitis; IgG4-related disease; Renal small-vessel vasculitis; Tubulointerstitial nephritis