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Fibrillary Glomerulonephritis and Monoclonal Gammopathy: Potential Diagnostic Challenges

Yi Da1, Giap Hean Goh2, Titus Lau1

  • 1Division of Nephrology, Department of Medicine, National University Hospital, Singapore, Singapore.

Frontiers in Oncology
|June 13, 2022
PubMed

Insights

Distinguishing fibrillary glomerulonephritis (FGN) with monoclonal gammopathy of renal significance (MGRS) from incidental monoclonal gammopathy of undetermined significance (MGUS) is challenging. Recent findings suggest MGRS is rare in FGN, necessitating advanced diagnostic approaches.

Area of Science:

  • Nephrology
  • Pathology
  • Immunology

Background:

  • Fibrillary glomerulonephritis (FGN) is a rare kidney disease characterized by abnormal fibrils.
  • Monoclonal gammopathy is present in up to 10% of FGN patients, complicating diagnosis.
  • Current methods for demonstrating monoclonality in FGN are limited by sensitivity and availability.

Purpose of the Study:

  • To address the diagnostic challenges in differentiating FGN with monoclonal gammopathy of renal significance (MGRS) from FGN with incidental monoclonal gammopathy of undetermined significance (MGUS).
  • To highlight the limitations of current diagnostic techniques for assessing monoclonality in FGN.
  • To emphasize the rarity of MGRS in FGN based on recent studies.

Main Methods:

  • Review of current diagnostic limitations including immunofluorescence in frozen tissue (IF-F) vs. paraffin sections (IF-P), immunoglobulin G (IgG) subtyping, and VH/VL domain monoclonality tests.
  • Consideration of DnaJ homolog subfamily B member 9 (DNAJB9) as a diagnostic marker for FGN.
  • Analysis of recent studies summarizing the incidence of MGRS in FGN.

Main Results:

  • Current kappa staining by immunofluorescence in frozen tissue (IF-F) shows suboptimal sensitivity compared to pronase-digested paraffin sections (IF-P).
  • Limitations exist in the availability of IgG subtyping and tests for VH/VL domain monoclonality in clinical settings.
  • Recent research indicates that genuine MGRS is extremely rare among FGN patients.

Conclusions:

  • Accurate differentiation between FGN-MGRS and FGN with incidental MGUS requires improved diagnostic strategies.
  • The discovery of DNAJB9 offers a potential diagnostic tool for FGN, reducing reliance on electron microscopy (EM).
  • Further research integrating advanced techniques like IF-P, IgG subtyping, VH/VL domain analysis, and DNAJB9 is crucial for understanding FGN and MGRS.
Keywords:
DnaJ homolog subfamily B member 9 (DNAJB9)dysproteinemiafibrillary glomerulonephritis (FGN)monoclonal gammopathymonoclonal gammopathy of renal significance (MGRS)

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