TY - JOUR
T1 - Recurrence of DNAJB9-Positive Fibrillary Glomerulonephritis After Kidney Transplantation
T2 - A Case Series
AU - El Ters, Mireille
AU - Bobart, Shane A.
AU - Cornell, Lynn D.
AU - Leung, Nelson
AU - Bentall, Andrew
AU - Sethi, Sanjeev
AU - Fidler, Mary
AU - Grande, Joseph
AU - Hernandez, Loren Herrera
AU - Cosio, Fernando G.
AU - Zand, Ladan
AU - Amer, Hatem
AU - Fervenza, Fernando C.
AU - Nasr, Samih H.
AU - Alexander, Mariam P.
N1 - Publisher Copyright:
© 2020
PY - 2020/10
Y1 - 2020/10
N2 - 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.
AB - 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.
KW - DnaJ homolog subfamily B member 9 (DNAJB9) staining
KW - Fibrillary glomerulonephritis (FGN)
KW - allograft kidney
KW - case series
KW - end-stage renal disease (ESRD)
KW - glomerular disease recurrence
KW - kidney transplantation
KW - monoclonal gammopathy
KW - protocol biopsy
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U2 - 10.1053/j.ajkd.2020.01.018
DO - 10.1053/j.ajkd.2020.01.018
M3 - Article
C2 - 32414663
AN - SCOPUS:85084664067
SN - 0272-6386
VL - 76
SP - 500
EP - 510
JO - American Journal of Kidney Diseases
JF - American Journal of Kidney Diseases
IS - 4
ER -