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Kidney Week

Abstract: TH-PO0442

From Gestational Tubulointerstitial Nephritis with Uveitis to Postpartum Atypical Anti-GBM Disease: A Rare Immunologic Association

Session Information

Category: Glomerular Diseases

  • 1401 Glomerular Diseases: Mechanisms, including Podocyte Biology

Authors

  • Kaur, Harpreet, UC Davis Health, Sacramento, California, United States
  • Kapa, Nandakishor, UC Davis Health, Sacramento, California, United States
  • Gupta, Rajib K., UC Davis Health, Sacramento, California, United States
  • Wiegley, Nasim, UC Davis Health, Sacramento, California, United States
Introduction

Tubulointerstitial nephritis with uveitis (TINU) syndrome is a rare entity characterized by tubulointerstitial nephritis without glomerular involvement. Anti-glomerular basement membrane (anti-GBM) disease, in contrast, targets the glomerular basement membrane, presents as rapidly progressive glomerulonephritis. We report a unique overlap of gestational TINU with biopsy-proven atypical anti-GBM disease postpartum.

Case Description

A 28-year-old woman at 30 weeks’ gestation presented with bilateral anterior granulomatous uveitis, acute kidney injury, and elevated urine beta-2 microglobulin, consistent with TINU syndrome. Serologic workup for other autoimmune conditions was negative. Kidney biopsy was deferred initially due to advanced pregnancy. Required temporary dialysis and recovered both kidney and ophthalmologic conditions with corticosteroid therapy, followed by uncomplicated vaginal delivery. Three months postpartum, developed sub-nephrotic proteinuria, hematuria, and mild AKI. Kidney biopsy demonstrated bright polyclonal linear IgG staining, mild mesangioproliferative changes, and ill-defined granulomas without crescents. Anti-GBM serologies remained negative on two occasions, consistent with atypical anti-GBM disease.

Discussion

To our knowledge, this is the first reported case of TINU syndrome associated with biopsy-proven atypical anti-GBM disease during pregnancy. In TINU, the primary autoantigen is modified C-reactive protein expressed in tubular and uveal tissue, whereas classical anti-GBM disease targets the alpha-3 chain of type IV collagen. Negative anti-GBM serologies suggest a distinct glomerular autoantigen, raising the possibility that epitope spreading from a tubular antigen drove cross-compartment autoimmunity. Pregnancy-related immune dysregulation and postpartum immune reconstitution may have further contributed.
Given mild histologic activity, cyclophosphamide and plasmapheresis were deferred in favor of mycophenolate mofetil, followed by successful steroid taper and stable renal function for the past 10 months. A high index of suspicion is needed for immune dysregulation during pregnancy and the postpartum period.