A case of ANCA-negative renal small-vessel vasculitis with tubulointerstitial infiltration of IgG4-positive plasma cells. Issue 5 (2nd September 2016)

• Record Type: Journal Article
• Authors: Sakairi, Toru; Okabe, Satoshi; Hiromura, Keiju; Motegi, Shinsuke; Sakurai, Noriyuki; Ikeuchi, Hidekazu; Kaneko, Yoriaki; Maeshima, Akito; Hirato, Junko; Nojima, Yoshihisa
• Is Part Of: Modern rheumatology. Volume 26:Issue 5(2016)
• Page Start: 780
• Page End: 783
• Publication Date: 2016-09-02
• Subjects: ANCA-associated vasculitis -- IgG4-related disease -- Renal small-vessel vasculitis -- Tubulointerstitial nephritis; Rheumatology -- Periodicals; 616.723005
• DOI: 10.3109/14397595.2014.915510
• Languages: English
• ISSNs: 1439-7595

Abstract

Abstract : 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.