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 McPhail, Nelson Leung

Research output: Contribution to journalArticle

1 Citation (Scopus)

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)
JournalAmerican Journal of Kidney Diseases
DOIs
StatePublished - Jan 1 2019

Fingerprint

Glomerulonephritis
Immunoglobulin Heavy Chains
Paraproteinemias
Kidney
HSP40 Heat-Shock Proteins
Heavy Chain Disease
Immunoglobulin G
Congo
Immunoglobulin Light Chains
Negative Staining
Kidney Diseases
Amyloidosis
Heat-Shock Proteins
Chronic Kidney Failure
Allografts
Autopsy
Biopsy
Light
Recurrence

Keywords

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

ASJC Scopus subject areas

  • Nephrology

Cite this

Nasr, S. H., Sirac, C., Bridoux, F., Javaugue, V., Bender, S., Rinsant, A., ... Leung, N. (2019). Heavy Chain Fibrillary Glomerulonephritis: A Case Report. American Journal of Kidney Diseases. https://doi.org/10.1053/j.ajkd.2019.01.032

Heavy Chain Fibrillary Glomerulonephritis : A Case Report. / Nasr, Samih H.; Sirac, Christophe; Bridoux, Frank; Javaugue, Vincent; Bender, Sebastien; Rinsant, Alexia; Kaaki, Sihem; Pinault, Emilie; Dasari, Surendra; Alexander, Mariam P; Said, Samar M.; Hogan, Jonathan J.; Dispenzieri, Angela; Touchard, Guy; McPhail, Ellen; Leung, Nelson.

In: American Journal of Kidney Diseases, 01.01.2019.

Research output: Contribution to journalArticle

Nasr, SH, Sirac, C, Bridoux, F, Javaugue, V, Bender, S, Rinsant, A, Kaaki, S, Pinault, E, Dasari, S, Alexander, MP, Said, SM, Hogan, JJ, Dispenzieri, A, Touchard, G, McPhail, E & Leung, N 2019, 'Heavy Chain Fibrillary Glomerulonephritis: A Case Report', American Journal of Kidney Diseases. https://doi.org/10.1053/j.ajkd.2019.01.032
Nasr, Samih H. ; Sirac, Christophe ; Bridoux, Frank ; Javaugue, Vincent ; Bender, Sebastien ; Rinsant, Alexia ; Kaaki, Sihem ; Pinault, Emilie ; Dasari, Surendra ; Alexander, Mariam P ; Said, Samar M. ; Hogan, Jonathan J. ; Dispenzieri, Angela ; Touchard, Guy ; McPhail, Ellen ; Leung, Nelson. / Heavy Chain Fibrillary Glomerulonephritis : A Case Report. In: American Journal of Kidney Diseases. 2019.
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