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Am J Kidney Dis. 2019 Apr 4. pii: S0272-6386(19)30162-3. doi: 10.1053/j.ajkd.2019.01.032. [Epub ahead of print]

Heavy Chain Fibrillary Glomerulonephritis: A Case Report.

Author information

1
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN. Electronic address: nasr.samih@mayo.edu.
2
Department of Immunology, National Center for Scientific Research, Joint Research Unit 7276, University of Limoges, Centre de référence de l'amylose AL et des autres maladies par dépôts d'immunoglobuline monoclonale, Limoges. Electronic address: christophe.sirac@unilim.fr.
3
Department of Nephrology, Dialysis and Renal Transplantation, University Hospital of Poitiers, Centre de référence de l'amylose AL et des autres maladies par dépôts d'immunoglobuline monoclonale, Poitiers.
4
Department of Immunology, National Center for Scientific Research, Joint Research Unit 7276, University of Limoges, Centre de référence de l'amylose AL et des autres maladies par dépôts d'immunoglobuline monoclonale, Limoges; Department of Nephrology, Dialysis and Renal Transplantation, University Hospital of Poitiers, Centre de référence de l'amylose AL et des autres maladies par dépôts d'immunoglobuline monoclonale, Poitiers.
5
Department of Immunology, National Center for Scientific Research, Joint Research Unit 7276, University of Limoges, Centre de référence de l'amylose AL et des autres maladies par dépôts d'immunoglobuline monoclonale, Limoges.
6
Department of Pathology, University Hospital of Poitiers, Poitiers, France.
7
University of Limoges, BISCEm Mass Spectrometry Platform, F-87000 Limoges, France.
8
Department of Health Sciences Research, Mayo Clinic, Rochester, MN.
9
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN.
10
Division of Nephrology, Department of Medicine, Hospital of the University of Pennsylvania, Philadelphia, PA.
11
Division of Hematology, Mayo Clinic, Rochester, MN.

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.

KEYWORDS:

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

PMID:
30955945
DOI:
10.1053/j.ajkd.2019.01.032

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