@article{a4265bf28b1e42b3b86c65377bdda8df,
title = "Heavy Chain Fibrillary Glomerulonephritis: A Case Report",
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",
author = "Nasr, {Samih H.} and Christophe Sirac and Frank Bridoux and Vincent Javaugue and Sebastien Bender and Alexia Rinsant and Sihem Kaaki and Emilie Pinault and Surendra Dasari and Alexander, {Mariam P.} and Said, {Samar M.} and Hogan, {Jonathan J.} and Angela Dispenzieri and Guy Touchard and McPhail, {Ellen D.} and Nelson Leung",
note = "Funding Information: Samih H. Nasr, MD, Christophe Sirac, PhD, Frank Bridoux, MD, PhD, Vincent Javaugue, MD, Sebastien Bender, MS, Alexia Rinsant, MS, Sihem Kaaki, MS, Emilie Pinault, MS, Surendra Dasari, PhD, Mariam P. Alexander, MD, Samar M. Said, MD, Jonathan J. Hogan, MD, Angela Dispenzieri, MD, Guy Touchard, MD, PhD, Ellen D. McPhail, MD, and Nelson Leung, MD. This work was supported in part by funding from Department of Laboratory Medicine and Pathology at the Mayo Clinic (Dr Nasr) and a grant from Ligue contre le cancer Limousin (Dr Sirac). The authors declare that they have no relevant financial interests. Received September 21, 2018. Evaluated by 3 external peer reviewers and a pathologist, with direct editorial input from an Associate Editor, who served as Acting Editor-in-Chief. Accepted in revised form January 21, 2019. The involvement of an Acting Editor-in-Chief was to comply with AJKD's procedures for potential conflicts of interest for editors, described in the Information for Authors & Journal Policies. Funding Information: This work was supported in part by funding from Department of Laboratory Medicine and Pathology at the Mayo Clinic (Dr Nasr) and a grant from Ligue contre le cancer Limousin (Dr Sirac). Publisher Copyright: {\textcopyright} 2019 National Kidney Foundation, Inc.",
year = "2019",
month = aug,
doi = "10.1053/j.ajkd.2019.01.032",
language = "English (US)",
volume = "74",
pages = "276--280",
journal = "American Journal of Kidney Diseases",
issn = "0272-6386",
publisher = "W.B. Saunders Ltd",
number = "2",
}