An abundance of IgG4 plasma cells is not specific for IgG4-related tubulointerstitial nephritis

IgG4-related tubulointerstitial nephritis (IgG4-TIN), the renal parenchymal lesion of IgG4-related sclerosing disease, is characterized, among other things, by the presence of numerous IgG4-positive plasma cells (IgG4PC) in the kidney infiltrate. The specificity of this finding for IgG4-TIN has not been addressed. To address this we examined 100 consecutive renal biopsy samples with active interstitial inflammation for the presence of IgG4PC, and correlated the findings with principal diagnosis, the available clinical histories, and the findings in four biopsy samples of IgG4-TIN. Eleven of the survey biopsy samples contained an average of more than 10 IgG4PC per × 200 field, including two with IgG4PC in numbers comparable to those in two of the IgG4-related tubulointerstitial disease biopsy samples. The principal pathological diagnoses in the IgG4PC-rich cases included anti-neutrophil cytoplasmic antibody-positive necrotizing glomerulonephritis (five cases), diabetic nephropathy (two cases), idiopathic interstitial nephritis (two cases), membranous glomerulonephritis (one case), and lupus nephritis (one case). There was no reason, based on histology or clinical history, to believe that any of these cases represented previously unsuspected IgG4-related tubulointerstitial disease. We conclude that the presence of numerous IgG4PC is essential to, but not sufficient for, the diagnosis of IgG4-TIN.