Case Series of IgG4-Related Tubulointerstitial Nephritis Resulting in Renal Failure or Renal Mass

Document Type

Conference Proceeding

Publication Date

11-2025

Publication Title

American Journal of Clinical Pathology

Abstract

Introduction/Objective: IgG4 related tubulointerstitial nephritis (TIN) is a form of autoimmune disease where IgG4-positive plasma cells infiltrate the kidney causing interstitial nephritis. This can present with either renal failure or a renal mass. This small series study presents the experience from our large university center. Methods/Case Report: We performed a retrospective analysis of 6 individuals with IgG4-TIN between 2017 and 2024. Results: 5 patients underwent biopsy and 1 underwent total nephrectomy. Ages ranged from 52 to 82 years; 3 females and 3 males. Three patients presented with renal masses ranging from 2.5 to 3 cm. Three patients’ kidneys were biopsied due to elevated serum creatinine ranging from 2.02 to 5.2 mg/dl. Light microscopy showed >10 IgG4 plasma cells/HPF or > 40% IgG4/IgG plasma cell ratio with storiform fibrosis and lymphocytes. 3/3 of patients who had immunofluorescence and electron microscopy showed positive IgG4 and electron dense deposits along tubular basement membrane (TBM). 4/4 were started on steroids. 2/3 showed treatment response to elevated baseline creatinine. Conclusion: Our data indicates that IgG4-TIN can present with either renal mass or renal dysfunction, supporting the view from other reports. A biopsy for uncertain renal mass is recommended to avoid total nephrectomy as seen in one of our cases.

Volume

164

Issue

Suppl 1

First Page

S165

Comments

American Society for Clinical Pathology (ASCP) Annual Meeting, November 17-20, 2025, Atlanta, GA

Last Page

S165

DOI

10.1093/ajcp/aqaf121.396

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