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

• Published in: American journal of kidney diseases Vol. 76; no. 4; pp. 500 - 510
• Main Authors: El Ters, Mireille, Bobart, Shane A., Cornell, Lynn D., Leung, Nelson, Bentall, Andrew, Sethi, Sanjeev, Fidler, Mary, Grande, Joseph, Hernandez, Loren Herrera, Cosio, Fernando G., Zand, Ladan, Amer, Hatem, Fervenza, Fernando C., Nasr, Samih H., Alexander, Mariam P.
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01-10-2020
• Subjects: allograft kidney; case series; DnaJ homolog subfamily B member 9 (DNAJB9) staining; end-stage renal disease (ESRD); Fibrillary glomerulonephritis (FGN); glomerular disease recurrence; kidney transplantation; monoclonal gammopathy; protocol biopsy; Fibrillary glomerulonephritis (FGN); glomerular disease recurrence; DnaJ homolog subfamily B member 9 (DNAJB9) staining; case series; monoclonal gammopathy; end-stage renal disease (ESRD); kidney transplantation; allograft kidney; protocol biopsy
• ISSN: 0272-6386; 1523-6838
• DOI: 10.1053/j.ajkd.2020.01.018

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.
Case series.
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.
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 243mg/d and 56mL/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.
Small study sample and shorter follow-up time in the nonrecurrent versus recurrent group.
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.