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Mod Rheumatol. 2014 May 20:1-4. [Epub ahead of print]

A case of ANCA-negative renal small-vessel vasculitis with tubulointerstitial infiltration of IgG4-positive plasma cells.

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  • 1Department of Medicine and Clinical Science, Gunma University Graduate School of Medicine , Maebashi, Gunma , Japan.


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.


ANCA-associated vasculitis; IgG4-related disease; Renal small-vessel vasculitis; Tubulointerstitial nephritis

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