DNAJB9 Is a Reliable Immunohistochemical Marker of Fibrillary Glomerulonephritis: Evaluation of Diagnostic Efficacy in a Large Series of Kidney Biopsies

Fibrillary glomerulonephritis (FGN) is a rare glomerular disease characterized by a challenging diagnostic workup requiring ultrastructural identification of 20 nm-thick randomly oriented fibrillar deposits. However, the recent introduction of DNAJB9 as a putative diagnostic marker of FGN could thor...

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Bibliographic Details
Published in:Biomedicines 2022-08, Vol.10 (9), p.2102
Main Authors: Gambella, Alessandro, Pitino, Chiara, Barreca, Antonella, Nocifora, Alberto, Giarin, Manuela Maria, Bertero, Luca, Biancone, Luigi, Roccatello, Dario, Papotti, Mauro, Cassoni, Paola
Format: Article
Language:English
Subjects:
Biopsy
DNAJB9
electron microscopy
fibrillary glomerulonephritis
fibrils
Glomerulonephritis
Hematuria
immunofluorescence
immunohistochemistry
Kidney diseases
Laboratories
Light
Microscopy
Pathology
Physiological aspects
Proteins
Stains & staining
Statistical analysis
Variables
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Summary:Fibrillary glomerulonephritis (FGN) is a rare glomerular disease characterized by a challenging diagnostic workup requiring ultrastructural identification of 20 nm-thick randomly oriented fibrillar deposits. However, the recent introduction of DNAJB9 as a putative diagnostic marker of FGN could thoroughly improve this diagnostic scenario. This study aims to assess the DNAJB9 immunohistochemical expression in a large series of FGN cases and to eventually confirm its role as a diagnostic marker of FGN. We evaluated the immunohistochemical expression of DNAJB9 (Rabbit Polyclonal, ThermoFisher) in a series of 77 FGN and 128 non-FGN cases diagnosed between January 1992 and June 2022 at the Pathology Unit of the AOU Città della Salute e della Scienza Hospital. DNAJB9 was expressed in 73 of the 74 evaluable FGN cases, mostly showing a strong glomerular positivity (68 cases). Additionally, DNAJB9 resulted positive in all challenging scenarios [early-stage (6), congophilic (4), combined (4), and uncertain (4) cases of FGN)]. DNAJB9 was negative in all non-FGN cases, eventually resulting in a specificity of 100% and sensitivity of 99%. In conclusion, we confirmed the role of DNAJB9 as a diagnostic marker of FGN. Its adoption in the clinical routine will allow a faster, more feasible, and more accurate FGN diagnosis.
ISSN:2227-9059
2227-9059
DOI:10.3390/biomedicines10092102