Immunoglobulin G4-related tubulointerstitial nephritis: A not to be missed diagnosis

Smita Mary Matthai, Anjali Mohapatra, Raiyani Palak, Gopal Basu

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Abstract

Immunoglobulin G4-related tubulointerstitial nephritis (IgG4-TIN) is a newly recognized clinicopathological entity characterized by a dense interstitial infiltrate of IgG4-positive plasma cells accompanied by fibrosis and obliterative phlebitis causing acute or chronic renal dysfunction amenable to corticosteroid therapy. IgG4-TIN is the dominant manifestation of renal involvement in IgG4-related disease (IgG4-RD) which is a novel, immune-mediated, fibroinflammatory and multiorgan disorder. We describe a case of IgG4-TIN with isolated renal involvement in an elderly male patient with poor response to corticosteroid therapy. The distinctive serological, histopathological, and ultrastructural features of this condition which can facilitate differential diagnosis of TIN are highlighted to emphasize the need for early diagnosis and preservation of kidney function.

Original languageEnglish
Pages (from-to)577-580
Number of pages4
JournalIndian Journal of Pathology and Microbiology
Volume60
Issue number4
DOIs
Publication statusPublished - Oct 2017
Externally publishedYes

Keywords

  • Corticosteroid therapy
  • immunoglobulin G4-related disease
  • tubulointerstitial nephritis

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