SUSTAINED RENAL RECOVERY IN IgG4-RELATED KIDNEY DISEASE AFTER DIALYSIS INITIATION: ROLE OF RITUXIMAB AND DECREMENTAL PERITONEAL DIALYSIS

 

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SUSTAINED RENAL RECOVERY IN IgG4-RELATED KIDNEY DISEASE AFTER DIALYSIS INITIATION: ROLE OF RITUXIMAB AND DECREMENTAL PERITONEAL DIALYSIS

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Ashan
Tuinder
Ashan Tuinder ashant@windowslive.com Bendigo Health Renal Bendigo Australia *
CINDY ONG CindyOng@bendigohealth.org.au Bendigo Health Renal Bendigo Australia -
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Introduction: IgG4-related kidney disease (IgG4-RKD) is a rare fibroinflammatory condition that can present along a spectrum of acute or chronic kidney injury. In severe cases, it may progress to end-stage kidney disease (ESKD) requiring dialysis. Glucocorticoid treatment is an effective induction therapy, however relapse and steroid toxicity remain major challenges. Early evidence supports rituximab as a potential adjunct, although data in IgG4-RKD remains limited.

Case Presentation: We report a 72-year-old male who presented with severe non-oliguric acute kidney injury (creatinine 663 μmol/L, eGFR 7 mL/min/1.73m²) due to IgG4-RKD. Kidney biopsy demonstrated extensive sclerotic interstitial fibrosis with tubular atrophy and a plasma cell rich inflammatory cell infiltrate, consistent with IgG4-RKD. His end stage kidney disease necessitated treatment with haemodialysis and subsequent transition to peritoneal dialysis (PD). His underlying disease was managed with prednisolone 40mg daily and 2 doses of rituximab 1g given 2 weeks apart. Despite the kidney biopsy findings, over subsequent months, his renal function steadily improved, allowing decremental PD prescription, followed by complete dialysis withdrawal at six months. He remains in remission on 6-monthly maintenance rituximab, off prednisolone, with eGFR 20 mL/min/1.73m² and sustained reduction in IgG4 levels.

Case report - for consideration of poster

This case demonstrates that meaningful renal recovery is possible in IgG4-RKD even after dialysis initiation and despite histopathological findings suggesting advanced disease. The case demonstrates two novel points: (1) rituximab may enable sustained remission and renal recovery, and (2) a decremental PD strategy provides a safe bridge to dialysis cessation in recovering kidney function. Further studies are required to identify predictors of renal recovery and to inform future guidelines for induction and maintenance therapy in IgG4-RKD.

Kewords