Please use this identifier to cite or link to this item: https://doi.org/10.3389/fonc.2022.880923
Title: Fibrillary Glomerulonephritis and Monoclonal Gammopathy: Potential Diagnostic Challenges
Authors: Da, Yi
Goh, Giap Hean
Lau, Titus
Chng, Wee Joo 
Soekojo, Cinnie Yentia
Keywords: Science & Technology
Life Sciences & Biomedicine
Oncology
fibrillary glomerulonephritis (FGN)
monoclonal gammopathy of renal significance (MGRS)
DnaJ homolog subfamily B member 9 (DNAJB9)
monoclonal gammopathy
dysproteinemia
B MEMBER 9
KIDNEY-TRANSPLANTATION
DNAJB9
IMMUNOFLUORESCENCE
OUTCOMES
DISEASE
Issue Date: 25-May-2022
Publisher: FRONTIERS MEDIA SA
Citation: Da, Yi, Goh, Giap Hean, Lau, Titus, Chng, Wee Joo, Soekojo, Cinnie Yentia (2022-05-25). Fibrillary Glomerulonephritis and Monoclonal Gammopathy: Potential Diagnostic Challenges. FRONTIERS IN ONCOLOGY 12. ScholarBank@NUS Repository. https://doi.org/10.3389/fonc.2022.880923
Abstract: Fibrillary glomerulonephritis (FGN) is a rare glomerular disease featured by the randomly arranged 12- to 24-nm fibrils under electron microscopy (EM). Up to 10% of FGN patients have monoclonal gammopathy. However, distinguishing between FGN as monoclonal gammopathy of renal significance (MGRS) and FGN from other causes with incidental monoclonal gammopathy of undetermined significance (MGUS) can be challenging, as the current way of demonstrating monoclonality is flawed due to (1) the suboptimal sensitivity of kappa staining by immunofluorescence in frozen tissue (IF-F) as compared to pronase-digested paraffin sections (IF-P), causing incorrect labeling of light chain restriction; (2) the unavailability of immunoglobulin G (IgG) subtyping in some centers; and (3) the unavailability of tests demonstrating the monoclonality of highly variable VH or VL domains in immunoglobulin structures in clinical use. The discovery of DnaJ homolog subfamily B member 9 (DNAJB9) allows diagnosis for FGN with less reliance on EM, and the summary of recent studies revealed that genuine MGRS is extremely rare among FGN. Further research integrating IF-P, IgG subtyping, VH or VL domain monoclonality confirmation, and DNAJB9 as diagnostic modalities, with corresponding clinical data including treatment response and prognosis, is required for a better understanding of this subject.
Source Title: FRONTIERS IN ONCOLOGY
URI: https://scholarbank.nus.edu.sg/handle/10635/228988
ISSN: 2234943X
DOI: 10.3389/fonc.2022.880923
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