Kidney Week

Abstract: FR-PO802

Prednisone Reduced Proteinuria and Stabilized Serum Creatinine in a Patient with Familial Fibronectin Glomerulopathy

Session Information

• Genetic Diseases of the Kidney - II
  November 08, 2019 | Location: Exhibit Hall, Walter E. Washington Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: Genetic Diseases of the Kidneys

• 1002 Genetic Diseases of the Kidneys: Non-Cystic

Authors

• Gray, Daniel A., University of Rochester, Rochester, New York, United States

Daniel A. Gray,

• Goldman, Bruce, University of Rochester, Rochester, New York, United States

Bruce Goldman,

• Gross, Matthew D., University of Rochester, Rochester, New York, United States

Matthew D. Gross,

Introduction

Fibronectin Glomerulopathy (FNG) is a rare, autosomal dominant disease characterized by proteinuria, hematuria and progressive renal failure associated with glomerular deposition of fibronectin, typically leading to ESRD in the 2^nd to 6^th decade. There is no established treatment for this condition beyond conservative measures such as blood pressure control and use of ACE inhibitors. We present a case of FNG associated with progressive CKD and nephrotic range proteinuria showing a sustained response to prednisone treatment.

Case Description

A 27 year old G₂P₂ female presented with 3 g of proteinuria, serum creatinine 0.7 mg/dl, inactive urinary sediment and normotension without medication. She was part of a large family with glomerular disease, including 3 members who died of cerebral hemorrhage or stroke in their thirties. The patient’s kidney biopsy showed mesangial deposition of fibronectin consistent with FNG. No interstitial fibrosis was seen. Genotyping revealed the Y973C fibronectin gene mutation. Despite maximal tolerable ACE inhibition, proteinuria increased to 4-6 g/g creat and serum creatinine increased to 1.0 mg/dl. Based on its use in IgA nephropathy, she was treated with prednisone 60 mg (~1 mg/Kg) for 2 mos, tapering by 20 mg every 2 mos. Proteinuria decreased to ~1 g/g creat for > 5 yrs and creatinine stabilized in the 1.2 mg/dl range with treatment. No significant side effects were encountered.

Discussion

Prednisone induced a sustained response in this patient. This is one of the first reports of effective treatment of FNG with immunosuppressive therapy. In conclusion, this protocol should be considered in FNG patients with nephrotic range proteinuria despite maximal ACE/ARB inhibition who have relatively preserved renal function.

Prednisone reduced proteinuria and stabilized serum creatinine