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Experimental Approaches for Biochemical Analysis of Glial Fibrillary Acidic Protein and Its Disease-associated Variants

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Fibronectin glomerulopathy.

Ikue Ishimoto¹, Eisei Sohara¹, Eisaku Ito²

¹Department of Nephrology, Graduate School of Medical and Dental Sciences , Tokyo Medical and Dental University , Tokyo , Japan.

Clinical Kidney Journal

|June 12, 2015

Summary

This case report highlights fibronectin glomerulopathy presenting atypically with rapid nephrotic syndrome in an elderly woman. Early consideration is crucial for timely diagnosis and management of this rare kidney disease.

Keywords:

fibronectin glomerulopathy haemodialysis nephrotic syndrome

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Area of Science:

Nephrology
Pathology

Background:

Fibronectin glomerulopathy (FG) typically presents in younger adults with slow progression.
It is characterized by fibronectin deposition in the glomeruli, leading to kidney dysfunction.

Purpose of the Study:

To report a rare case of rapidly progressive nephrotic syndrome due to FG in an elderly patient.
To emphasize the importance of considering FG in the differential diagnosis of nephrotic syndrome in older individuals.

Main Methods:

Case presentation of a 78-year-old woman with rapid onset nephrotic syndrome.
Analysis of renal biopsy findings including light microscopy, electron microscopy, and immunohistochemistry for fibronectin.

Main Results:

The patient exhibited rapid increase in proteinuria (6.8 g/day) and microscopic hematuria.
Renal biopsy revealed lobular glomerulopathy and MPGN-like lesions with granular deposits and fibril formation.
Immunohistochemistry confirmed intense fibronectin staining in the mesangium and peripheral loops.

Conclusions:

This case demonstrates an atypical, rapid presentation of fibronectin glomerulopathy in an elderly patient.
Fibronectin glomerulopathy should be considered in the differential diagnosis of nephrotic syndrome, especially in older populations, despite its typical slower progression.