TIN IgG4-RKD can be confirmed by the presence of tubulointerstitial nephritis with >10 IgG4+ plasma cells/hpf in the most concentrated field, plus at least one of the following: | |
• Histology: | |
  ο Tubular basement membrane immune complex deposits by immunofluorescence, immunohistochemistry, and/or electron microscopy | |
• Imaging: | |
  ο Small peripheral low-attenuation cortical nodules, round or wedge-shaped lesions, or diffuse patchy involvement | |
• Serology: | |
  ο Elevated serum IgG4 or total IgG level | |
• Other organ involvement such as the following: | |
  ο AIP, sclerosing cholangitis, inflammatory masses of any origin, sialadenitis, inflammatory aortic aneurysm, lung involvement, retroperitoneal fibrosis |