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Practice patterns and outcomes for pediatric ANCA vasculitis transplant recipients in a national cohort

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Abstract

Background

Anti-neutrophil cytoplasmic autoantibody (ANCA) vasculitis is a rare condition in pediatric patients. Little is known about practice patterns and outcomes of pediatric transplant patients. The purpose of our study was to examine differences in patient characteristics, immunosuppression, and long-term graft outcomes between ANCA and non-ANCA vasculitis recipients.

Methods

We used the Scientific Registry of Transplant Recipients to evaluate pediatric ANCA vasculitis recipients between the ages of 1 and 22 years old from 1991 to 2017 and compared them to non-ANCA vasculitis patients during the same time cohort in the USA.

Results

A total of 26,431 transplant recipients were identified, of these, 337 with ANCA vasculitis. Mean 1-year eGFR was 62.46 and 64.92 ml/min/1.73 m2 (p = 0.002), and mean 5-year eGFR was 57.95 and 59.38 ml/min/1.73 m2 (p = 0.18) between the non-ANCA and ANCA groups, respectively. Five-year graft survival was similar in both groups (non-ANCA 75.5 vs. ANCA 78.6%; p = 0.19). Of those with graft loss within the ANCA group, only 0.6% was secondary to disease recurrence (p = 0.14).

Conclusions

Kidney transplant is a safe treatment modality for children with ANCA-related kidney failure. ANCA patients have comparable graft survival when compared to the general transplant population with a low risk of recurrence. Thymoglobulin was used in a higher proportion within the ANCA group compared to the non-ANCA group. Tacrolimus, mycophenolate mofetil/mycophenolic acid, and steroids were the predominant maintenance immunosuppression used in both groups.

Graphical abstract

A higher resolution version of the Graphical abstract is available as Supplementary information.

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Data Availability

Data from SRTR. Please see disclaimer below.

Code availability

SPSS version 26 (IBM Inc., Armonk, NY, USA).

Abbreviations

ANCA:

Anti-neutrophil cytoplasmic autoantibody

GPA:

Granulomatosis with polyangiitis

MPA:

Microscopic polyangiitis

EGPA:

Eosinophilic granulomatosis with polyangiitis

CKD 5:

Stage 5 chronic kidney disease

SRTR:

Scientific Registry of Transplant Recipients

OPTN:

Organ Procurement and Transplantation Network

KDPI:

Kidney donor profile index

HLA:

Human leukocyte antigen

PRA:

Panel reactive antibodies

eGFR:

Estimated glomerular filtration rate

KDIGO:

Kidney Disease: Improving Global Outcomes

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Author information

Authors and Affiliations

  1. Maria Fareri Children’s Hospital, Westchester Medical Center, New York Medical College, Valhalla, NY, USA

    Stella Kilduff

  2. Department of Surgery, Clinical Research Unit, Westchester Medical Center, Valhalla, NY, USA

    Jonathan Butler

  3. Department of Surgery, Division of Intra-Abdominal Transplant & Hepatobiliary Surgery, Westchester Medical Center, New York Medical College, Valhalla, NY, USA

    Hiroshi Sogawa

  4. Maria Fareri Children’s Hospital, Westchester Medical Center, Boston Children’s Health Physicians, New York Medical College, Valhalla, NY, USA

    Sonia Solomon

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  1. Stella Kilduff
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  2. Jonathan Butler
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  4. Sonia Solomon
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Contributions

Stella Kilduff, MD: participated in research design and participated in the writing of the paper and review of the paper.

Jonathan Butler, MS: participated in data analysis, contributed new reagents or analytic tools, and participated in the performance of the research and review of the paper.

Hiroshi Sogawa, MD: participated in research design and review of the paper.

Sonia Solomon, DO: participated in research design, participated in the performance of the research, and participated in the writing of the paper and review of the paper.

Corresponding author

Correspondence to Sonia Solomon.

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The authors declare no competing interests.

Disclaimer

The data reported here have been supplied by the Hennepin Healthcare Research Institute (HHRI) as the contractor for the Scientific Registry of Transplant Recipients (SRTR). The interpretation and reporting of these data are the responsibility of the author(s) and in no way should be seen as an official policy of or interpretation by the SRTR or the US Government.

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Kilduff, S., Butler, J., Sogawa, H. et al. Practice patterns and outcomes for pediatric ANCA vasculitis transplant recipients in a national cohort. Pediatr Nephrol 37, 1667–1673 (2022). https://doi.org/10.1007/s00467-021-05341-z

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  • DOI: https://doi.org/10.1007/s00467-021-05341-z

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