Heavy Chain Fibrillary Glomerulonephritis: A Case Report

Samih H. Nasr, Christophe Sirac, Frank Bridoux, Vincent Javaugue, Sebastien Bender, Alexia Rinsant, Sihem Kaaki, Emilie Pinault, Surendra Dasari, Mariam P. Alexander, Samar M. Said, Jonathan J. Hogan, Angela Dispenzieri, Guy Touchard, Ellen D. McPhail, Nelson Leung

Research output: Contribution to journalArticle

6 Scopus citations

Abstract

Heavy chain amyloidosis and heavy chain deposition disease are the only known kidney diseases caused by the deposition of truncated immunoglobulin heavy chains. Fibrillary glomerulonephritis typically results from deposition of DNAJB9 (DnaJ heat shock protein family [Hsp40] member B9) and polytypic immunoglobulin G (IgG). We describe a patient with monoclonal gammopathy (IgG with λ light chain) who developed DNAJB9-negative fibrillary glomerulonephritis leading to end-stage kidney disease, with recurrence in 2 kidney allografts. Pre- and postmortem examination showed glomerular deposition of Congo red–negative fibrillar material that was determined to be immunoglobulin heavy chain. We propose the term “heavy chain fibrillary glomerulonephritis” to describe this lesion, which appears to be a rare kidney complication of monoclonal gammopathy. The diagnosis should be suspected when the kidney biopsy shows fibrillary glomerulonephritis with negative staining for immunoglobulin light chains and DNAJB9; the diagnosis can be confirmed using immunochemical and molecular studies.

Original languageEnglish (US)
Pages (from-to)276-280
Number of pages5
JournalAmerican Journal of Kidney Diseases
Volume74
Issue number2
DOIs
StatePublished - Aug 2019

Keywords

  • Fibrillary glomerulonephritis
  • case report
  • fibrils
  • heavy chain fibrillary glomerulonephritis
  • heavy chain sequencing
  • immunoglobulin heavy chain
  • kidney biopsy
  • monoclonal gammopathy

ASJC Scopus subject areas

  • Nephrology

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  • Cite this

    Nasr, S. H., Sirac, C., Bridoux, F., Javaugue, V., Bender, S., Rinsant, A., Kaaki, S., Pinault, E., Dasari, S., Alexander, M. P., Said, S. M., Hogan, J. J., Dispenzieri, A., Touchard, G., McPhail, E. D., & Leung, N. (2019). Heavy Chain Fibrillary Glomerulonephritis: A Case Report. American Journal of Kidney Diseases, 74(2), 276-280. https://doi.org/10.1053/j.ajkd.2019.01.032