Laboratory Medicine Program

Fibrillary Glomerulonephritis Confirmation
(FGN)

Clinical Decription:
Fibrillary glomerulonephritis (FGN) is a rare kidney disease with fibrillary deposits in the glomeruli that contain polyclonal IgG and complement, indicating immune complex deposition. Although usually Congo-red negative, recently cases with weak Congo-red positivity have been observed, making the distinction from amyloid more challenging. Liquid chromatography-tandem mass spectrometry (LC-MS/MS) performed on microdissected glomeruli from patients with FGN demonstrates a unique proteomic profile including the protein DNAJB9 (Dasari S et al: DnaJ Heat Shock Protein Family B Member 9 is a Novel Biomarker for Fibrillary GN. J Am Soc Nephrol 2018;(1):51-56). The presence of DNAJB9 was found to be highly sensitive and specific for FGN, distinguishing it from other glomerular diseases including amyloid, immunotactoid glomerulopathy, and immune complex-mediated proliferative glomerulonephritis. The presence of DNAJB9, in the appropriate clinical and pathological context, can be useful to establish a diagnosis of FGN.

Method: LC-MS/MS

Component Tests Used: n/a

Reference Ranges Used:
An interpretation will be provided.

Specimen Type: paraffin-embedded tissue blocks
Volume: n/a

Shipping: N/A

Special Instructions: More Information: Call 14-4560 (Dr. John) or 14-8589 (Dr. Kulasingam)

Testing Schedule(s): Biweekly.

Turnaround Time: 2-3 weeks

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For more information, call 416.340.5227 or 1.866.865.5227

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