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Abstract: FR-PO771

IgG4-Related Disease Tubulointerstitial Nephritis: Successful Treatment of Post-Transplant Relapse

Session Information

Category: Transplantation

  • 2102 Transplantation: Clinical


  • Earl, Wesley Joshua, Columbia University Irving Medical Center, New York, New York, United States
  • Dube, Geoffrey K., Columbia University Irving Medical Center, New York, New York, United States
  • Batal, Ibrahim, Columbia University Irving Medical Center, New York, New York, United States

IgG4-related disease (RD) is a heterogenous condition with renal manifestations. We report a case of IgG4-RD tubulointerstitial nephritis (TIN) with relapse post-transplant.

Case Description

A 66 yo male with a history of chronic kidney disease (CKD) 3b presents with acute renal failure. He underwent a renal biopsy demonstrating interstitial infiltrate with IgG4+ plasma cells consistent with TIN and was diagnosed with IgG4-RD.

He underwent preemptive live donor kidney transplant without complication and was started on tacrolimus and mycophenolate (MMF). Two years post-transplant creatinine increased from 1.0 to 1.5. Allograft biopsy showed IgG4+ plasma cells consistent with TIN from recurrence of IgG4-RD. He started on prednisone and rituximab with improvement of his creatinine to 1.2. Steroids were tapered over months. Creatinine returned to 1.1. Ten months after rituximab repeat allograft biopsy showed improvement of TIN . He required no further doses of rituximab and remains on prednisone, tacrolimus and MMF. 11-years after transplantation his creatinine remains 1.1.


IgG4-RD is characterized by autoimmune dysregulation of IgG4+ plasmacytes impacting many organs in an inflammatory to fibrotic process. Th cells stimulate B cells to produce IgG4 and collectively release inflammatory cytokines. Fibroblasts replace the inflammatory milieu with stromal tissue. IgG4 antibodies play an unknown role.

Renal involvement can be intrinsic or secondary to retroperitoneal fibrosis. Intrinsic disease typically manifests as TIN. Laboratory findings of TIN include pyuria, variable proteinuria, hypocomplementemia and elevated IgG4 subclass. Histologic evaluation demonstrates IgG4+ plasmacyte infiltrate, storiform fibrosis, and obliterative phlebitis. Progression to renal failure is uncommon and kidney transplant occurs rarely.

Treatment of IgG4-RD includes glucocorticoids tapered over 6 months. Rituximab is used with 90-100% remission rate even if refractory to treatment and relapse. Recurrent IgG4-RD TIN post-transplant has only been reported once prior in literature. This is the first reported case without concurrent rejection and the first case demonstrating successful treatment of recurrent IgG4-RD post-transplant. Our case suggests effective use of rituximab, glucocorticoids, and MMF for recurrent IgG4-RD TIN in a post-transplant patient.