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Abstract: PO1897

Recurrent Fibrillary Glomerulonephritis Secondary to Chronic Lymphocytic Leukemia: A Rare Case of Treatment Success

Session Information

Category: Onco-Nephrology

  • 1500 Onco-Nephrology

Author

  • Shah, Rafeea, University Hospitals Birmingham NHS Foundation Trust, Birmingham, Birmingham, United Kingdom
Introduction

Fibrillary GN is a rare form of glomerular disease characterized by the random deposition of small (20nm) fibrils in the mesangium and capillary walls of the glomerulus. There is a recognized association with hepatitis C, malignancy and autoimmune conditions. It is poorly responsive to treatment and up to half of the patients reach ESRD by six years. We present a case of Fibrillary GN secondary to CLL with complete remission after receiving Bendamustine and Rituximab and a subsequent relapse treated with Ibrutinib with good renal recovery.

Case Description

A 69-year-old male presented in 2014 with a pathological spinal fracture and was subsequently found to have CLL. He had normal renal function and no proteinuria at the time of diagnosis and his CLL was managed conservatively. In 2016 he developed nephrotic syndrome (ACR >1000) with a significant decline in renal function to an eGFR of 18ml/min. He underwent a renal biopsy which showed an MPGN pattern of injury with fibrils on electron microscopy suggesting fibrillary glomerulonephritis. He received Bendamustine and Rituximab for CLL and went into complete remission of his nephrotic syndrome and improvement in renal function to eGFR of 57ml/min. He remained in remission for four years. In 2020 he had relapse of his nephrotic syndrome and eGFR dropped to 17ml/min. Further imaging suggested progression of the CLL. He had a further renal biopsy which again confirmed recurrent Fibrillary GN. He was started on the Tyrosine kinase inhibitor (TKI) Ibrutinib in December 2020 and within 3 months his renal function had improved to an eGFR of 33ml/min and reduction in proteinuria.

Discussion

TKIs have mostly been linked with kidney injury secondary to the potential deleterious effects on the renal endothelium. This is the first reported case of the use of a TKI as a treatment for Fibrillary GN secondary to CLL. In addition, there is scarcity of experience with relapse of Fibrillary GN as it is usually a progressive disease and with little prospect of recovery. This case highlights the following:
If there is an identifiable cause driving Fibrillary GN, treatment can be associated with remission of proteinuria and improvement in renal function.
Monitoring of the underlying disease is important as recurrence can result in subsequent relapse of nephrotic syndrome.
TKIs used with caution can be beneficial in the setting of MGRS.