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Outcome of kidney transplantation in pediatric patients with ANCA-associated glomerulonephritis: a single-center experience

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Abstract

Objectives

Data on kidney transplant outcomes for pediatric patients with end-stage renal disease (ESRD) secondary to anti-neutrophil cytoplasmic antibody glomerulonephritis (ANCA GN), particularly granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), is limited. We describe our experience of kidney transplantation in pediatric ANCA GN patients.

Methods

We performed a retrospective review of patients with ANCA GN who developed ESRD and were transplanted at a single center between the years 2000 and 2014.

Results

Since 2000, there were seven pediatric patients with ANCA GN (four MPA) transplanted. Mean age at ANCA GN diagnosis was 11.8 ± 2.8 (range, 7.2–15.4) years. All seven were ANCA (three anti-PR3/four anti-MPO) positive. Estimated glomerular filtration rate (eGFR) at diagnosis was 11.7 ± 6.3 ml/min/1.73 m2. All received steroids and cyclophosphamide and three (23.3%) received plasma exchange. Six were dialysis dependent by 6 months post diagnosis. Time from diagnosis to transplant was 30 ± 12 (range, 17–48) months. Six of the seven received a deceased donor transplant. All patients received induction therapy and standard maintenance immunosuppression post transplant. Median duration of follow-up post transplantation was 27 months (range, 13–88 months). Median eGFR at last follow-up was 77 ml/min/1.73 m2 (range, 7.9–83.5). One patient lost her transplant to acute cellular rejection following non-adherence to immunosuppression after 21 months of stable transplant function. No patient had recurrence of vasculitis, either renal or extra-renal.

Conclusions

Short-term patient and allograft survival in pediatric patients with ESRD secondary to ANCA GN seems excellent, with no recurrence of vasculitis post transplant in this small cohort.

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Authors and Affiliations

  1. Division of Nephrology, The Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada

    Damien Noone & Diane Hebert

  2. Department of Paediatrics, University of Toronto, Toronto, Ontario, Canada

    Damien Noone, Rae S. M. Yeung & Diane Hebert

  3. Division of Rheumatology, The Hospital for Sick Children, Toronto, Ontario, Canada

    Rae S. M. Yeung

  4. Department of Immunology, University of Toronto, Toronto, Ontario, Canada

    Rae S. M. Yeung

Authors
  1. Damien Noone
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  2. Rae S. M. Yeung
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Correspondence to Damien Noone.

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Ethics approval was obtained from the Research Ethics Board at The Hospital for Sick Children.

Conflict of interest

DN, RSMY, or DH have no conflicts of interest.

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Noone, D., Yeung, R.S.M. & Hebert, D. Outcome of kidney transplantation in pediatric patients with ANCA-associated glomerulonephritis: a single-center experience. Pediatr Nephrol 32, 2343–2350 (2017). https://doi.org/10.1007/s00467-017-3749-6

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  • DOI: https://doi.org/10.1007/s00467-017-3749-6

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