Differential Diagnosis of IgG4-Related Tubulointerstitial Nephritis: An Overview

  • Yutaka Yamaguchi
  • Satoshi Hara
  • Mitsuhiro Kawano


A variety of diseases cause tubulointerstitial inflammation in the basic structure of the tubulointerstitium. The histological variation differs according to the onset (acute or chronic) and type of immune mechanism (primary or secondary), and some specific findings are of help in surmising the etiology of tubulointerstitial nephritis (TIN).

The histopathological picture in IgG4-related TIN is mixed: some patients present with acute, and others slowly progressive renal insufficiency. IgG4-related TIN is classified appropriately as a secondary cause of TIN because it occurs not in association with a primary glomerular disease but rather as a manifestation of a systemic underlying condition, admixed with tubulointerstitial immune deposition. Although IgG4-related TIN reveals some specific pathological hallmarks, pathologists should differentiate some diseases such as idiopathic hypocomplementemic TIN, nephropathy associated with the diffuse infiltrative lymphocytosis syndrome, Epstein-Barr virus infection of renal proximal tubule cells, predominantly tubulointerstitial lupus nephritis, multicentric Castleman disease, and kidney infiltration by lymphoproliferative disorders.

In this chapter, the basic structure of the tubulointerstitium, various aspects of TIN and its pathology, and the differential diagnosis of IgG4-related TIN are focused on.


Lupus Nephritis Interstitial Cell Tubulointerstitial Nephritis Tubular Basement Membrane Peritubular Capillary 
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Copyright information

© Springer Japan 2016

Authors and Affiliations

  • Yutaka Yamaguchi
    • 1
  • Satoshi Hara
    • 2
  • Mitsuhiro Kawano
    • 2
  1. 1.Yamaguchi’s Pathology LaboratoryChibaJapan
  2. 2.Division of Rheumatology, Department of Internal MedicineKanazawa University HospitalKanazawaJapan

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