Recurrence of DNAJB9-Positive Fibrillary Glomerulonephritis After Kidney Transplantation: A Case Series

Mireille El Ters, Shane A. Bobart, Lynn D. Cornell, Nelson Leung, Andrew Bentall, Sanjeev Sethi, Mary Fidler, Joseph Grande, Loren Herrera Hernandez, Fernando G. Cosio, Ladan Zand, Hatem Amer, Fernando C. Fervenza, Samih H. Nasr, Mariam P. Alexander

Research output: Contribution to journalArticlepeer-review

1 Scopus citations

Abstract

Rationale & Objective: Fibrillary glomerulonephritis (FGN) is a rare glomerular disease that often progresses to kidney failure requiring kidney replacement therapy. We have recently identified a novel biomarker of FGN, DnaJ homolog subfamily B member 9 (DNAJB9). In this study, we used sequential protocol allograft biopsies and DNAJB9 staining to help characterize a series of patients with native kidney FGN who underwent kidney transplantation. Study Design: Case series. Setting & Participants: Between 1996 and 2016, kidney transplantation was performed on 19 patients with a reported diagnosis of FGN in their native/transplant kidneys. Using standard diagnostic criteria and DNAJB9 staining, we excluded 5 patients (4 atypical cases diagnosed as possible FGN and 1 donor-derived FGN). Protocol allograft biopsies had been performed at 4, 12, 24, 60, and 120 months posttransplantation. DNAJB9 immunohistochemistry was performed using an anti-DNAJB9 rabbit polyclonal antibody. Pre- and posttransplantation demographic and clinical characteristics were collected. Summary statistical analysis was performed, including nonparametric statistical tests. Observations: The 14 patients with FGN had a median posttransplantation follow-up of 5.7 (IQR, 2.9-13.8) years. 3 (21%) patients had recurrence of FGN, detected on the 5- (n = 1) and 10-year (n = 2) allograft biopsies. Median time to recurrence was 10.2 (IQR, 5-10.5) years. Median levels of proteinuria and iothalamate clearance at the time of recurrence were 243 mg/d and 56 mL/min. The remaining 11 patients had no evidence of histologic recurrence on the last posttransplantation biopsy, although the median time of follow-up was significantly less at 4.4 (IQR, 2.9-14.4) years. 3 (21%) patients had a monoclonal protein detectable in serum obtained pretransplantation; none of these patients had recurrent FGN. Limitations: Small study sample and shorter follow-up time in the nonrecurrent versus recurrent group. Conclusions: In this series, FGN had an indolent course in the kidney allograft in that detectable histologic recurrence did not appear for at least 5 years posttransplantation.

Original languageEnglish (US)
Pages (from-to)500-510
Number of pages11
JournalAmerican Journal of Kidney Diseases
Volume76
Issue number4
DOIs
StatePublished - Oct 2020

Bibliographical note

Funding Information:
Funding for this project was made possible by Mayo Clinic Anatomic Pathology funds.

Keywords

  • DnaJ homolog subfamily B member 9 (DNAJB9) staining
  • Fibrillary glomerulonephritis (FGN)
  • allograft kidney
  • case series
  • end-stage renal disease (ESRD)
  • glomerular disease recurrence
  • kidney transplantation
  • monoclonal gammopathy
  • protocol biopsy

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