Abstract
Glomerulopathy with Fibronectin Deposits (GFND) is a rare, autosomal dominant disease characterized by proteinuria, hematuria and progressive renal failure associated with glomerular deposition of fibronectin, frequently resulting in end-stage renal disease (ESRD). There is no established treatment for this condition beyond conservative measures such as blood pressure control and the use of angiotensin-converting enzyme (ACE) inhibitors. We present a case of GFND associated with progressive chronic kidney disease (CKD) and nephrotic range proteinuria showing a sustained response to prednisone treatment. A 27-year-old G2P2 Caucasian female presented with 3 g/day of proteinuria, serum creatinine (Cr) 0.7 mg/dL, inactive urinary sediment and normotension without medication. She was part of a large family with glomerular disease, including three members who died of cerebral hemorrhage or stroke in their thirties. The patient’s kidney biopsy showed mesangial deposition of fibronectin consistent with GFND. No interstitial fibrosis was seen. Genotyping revealed the Y973C FN1 gene mutation. Despite maximal tolerable ACE inhibition, proteinuria increased to 4–6 g/g Cr and serum Cr increased to 1.0 mg/dL. She was treated with prednisone 60 mg (~ 1 mg/Kg) daily for 2 mos and then tapered by ~ 0.2 mg/Kg every month for 6 mos of total therapy. Proteinuria decreased to ~ 1 g/g Cr for > 5 years and serum Cr stabilized in the 1.2 mg/dL range with treatment. No significant side effects were encountered. In conclusion, this protocol should be considered in GFND patients with nephrotic range proteinuria despite maximal angiotensin system inhibition who have relatively preserved renal function.
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Acknowledgements
The authors would like to thank family members for their helpfulness in providing clinical history and family contact information necessary to put together the pedigree (Fig. 1). We would also like to thank Sierra Kovar and Karen Vanderbilt (Pathology and Laboratory Medicine, University of Rochester) as well as Chris Larsen (Arkana Laboratories, Little Rock, AR) for technical assistance with the fibronectin immunohistochemistry.
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Goldman, B.I., Panner, B.J., Welle, S.L. et al. Prednisone-induced sustained remission in a patient with familial fibronectin glomerulopathy (GFND). CEN Case Rep 10, 510–514 (2021). https://doi.org/10.1007/s13730-021-00595-w
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DOI: https://doi.org/10.1007/s13730-021-00595-w