Case Report

IgG4-Related Kidney Disease: Report of a Case Presenting as a Renal Mass

Table 2

Diagnostic criteria for IgG4-related tubulointerstitial nephritis (TIN) proposed by Raissian et al. [10].

HistologyPlasma cell-rich tubulointerstitial nephritis with >10 IgG4+ plasma cells/HPF in the most concentrated
Tubular basement membrane immune complex deposits by immunofluorescence, immunohistochemistry, and/or electron

ImagingSmall peripheral low-attenuation cortical nodules, round or wedge-shaped lesions, or diffuse patchy involvement
Diffuse marked enlargement of kidneys

SerologyElevated serum IgG4 or total IgG level

Other organs involvementIncluding autoimmune pancreatitis, sclerosing cholangitis, inflammatory masses in any organ, sialadenitis, inflammatory aortic aneurysm, lung involvement, and retroperitoneal fibrosis

Diagnosis of IgG4-TIN requires the histologic feature of plasma cell-rich TIN with increased IgG4+ plasma cells and at least one other feature from the categories of “imaging,” “serology,” or “other organ involvement”; mandatory criterion; supportive criterion, present in >80% of cases.