Although tubulointerstitial nephritis with IgG4+ plasma cell (PC) infiltration is a hallmark of IgG4-related kidney disease (IgG4-RKD), only a few studies are available about the minimum number of IgG4+？PC needed for diagnosis along with IgG4+/IgG+？PC ratio in the kidney. In addition, the significance of the deposition of IgG or complement as a reflection of humoral immunity involvement is still uncertain. In this study, we analyzed 20 Japanese patients with IgG4-RKD to evaluate the number of IgG4+？PCs along with IgG4+/IgG+？PC ratio and involvement of humoral immunity. The average number of IgG4+？PCs was 43.8/hpf and the average IgG4+/IgG+ or IgG4+/CD138+ ratio was 53%. IgG and C3 granular deposits on the tubular basement membrane (TBM) were detected by immunofluorescence microscopy in 13% and 47% of patients, respectively. Nine patients had a variety of glomerular lesions, and 7 of them had immunoglobulin or complement deposition in the glomerulus. In conclusion, we confirmed that infiltrating IgG4+？PCs > 10/hpf and/or IgG4/IgG (CD138)+？PCs > 40% was appropriate as an item of the diagnostic criteria for IgG4-RKD. A relatively high frequency of diverse glomerular lesions with immunoglobulin or complement deposits and deposits in TBM may be evidence of immune complex involvement in IgG4-related disease. 1. Introduction The main histopathological finding in the kidney of IgG4-RD is tubulointerstitial nephritis (TIN) [1–3], which may result in renal failure [4]. IgG4-related TIN is composed of dense lymphoplasmacytic infiltrates with fibrosis and copious IgG4+ plasma cell infiltration, which are common features shared by other involved organs [5], and these common pathologic features in the kidney have clearly been described by previous studies [1–3]. However, the minimum number of IgG4+ plasma cells needed for diagnosis has been differently reported in each affected organ [6–9], and only a few studies are available about the actual number of IgG4+ plasma cells evaluated along with IgG4+/IgG+ plasma cell ratio in IgG4-related kidney disease (IgG4-RKD) [2]. In addition to this issue, case reports or case series of a variety of glomerular disease concurrent with TIN have been accumulated [10–26]. These glomerular lesions are frequently accompanied by immunoglobulin or complement deposits suggesting that immune complexes might be involved in the pathogenesis of some cases with IgG4-RKD [2, 3]. However, the significance of these glomerular lesions as a reflection of humoral immunity involvement is still uncertain, and whether these glomerular lesions represent
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