TY - JOUR
T1 - IgG4-related tubulointerstitial nephritis with membranous nephropathy
AU - Fervenza, Fernando C.
AU - Downer, Gregory
AU - Beck, Laurence H.
AU - Sethi, Sanjeev
PY - 2011/8
Y1 - 2011/8
N2 - We describe a 67-year-old woman who presented with significant proteinuria and hematuria. Kidney biopsy showed immunoglobulin G4 (IgG4)-related tubulointerstitial nephritis (TIN) with concurrent membranous nephropathy. IgG4-related TIN is a recently described entity that presents with progressive decreased kidney function and is characterized by a plasma cellrich infiltrate that is positive for IgG4. It is associated with patchy, often well-localized, tubular atrophy and interstitial fibrosis. Workup for circulating antiphospholipase A2 receptor antibodies was negative, suggesting that the membranous nephropathy was not "primary" and may be linked to the IgG4-related disease. The presence of significant proteinuria and hematuria in the setting of IgG4-related TIN should raise suspicion of a glomerular disease. It is important to correctly diagnose IgG4-related TIN and concurrent membranous nephropathy because the lesion responds well to steroid therapy.
AB - We describe a 67-year-old woman who presented with significant proteinuria and hematuria. Kidney biopsy showed immunoglobulin G4 (IgG4)-related tubulointerstitial nephritis (TIN) with concurrent membranous nephropathy. IgG4-related TIN is a recently described entity that presents with progressive decreased kidney function and is characterized by a plasma cellrich infiltrate that is positive for IgG4. It is associated with patchy, often well-localized, tubular atrophy and interstitial fibrosis. Workup for circulating antiphospholipase A2 receptor antibodies was negative, suggesting that the membranous nephropathy was not "primary" and may be linked to the IgG4-related disease. The presence of significant proteinuria and hematuria in the setting of IgG4-related TIN should raise suspicion of a glomerular disease. It is important to correctly diagnose IgG4-related TIN and concurrent membranous nephropathy because the lesion responds well to steroid therapy.
KW - Immunoglobulin G4 (IgG4)-related tubulointerstitial nephritis (TIN)
KW - immunoglobulin G4 (IgG4)-related sclerosing disease
KW - membranous nephropathy
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U2 - 10.1053/j.ajkd.2011.05.006
DO - 10.1053/j.ajkd.2011.05.006
M3 - Article
C2 - 21705127
AN - SCOPUS:79960846722
SN - 0272-6386
VL - 58
SP - 320
EP - 324
JO - American Journal of Kidney Diseases
JF - American Journal of Kidney Diseases
IS - 2
ER -