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Paediatric Rheumatology

 

Wide variation in glucocorticoid dosing in paediatric ANCA-associated vasculitis with renal disease: a paediatric vasculitis initiative study


1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23

 

  1. BC Children's Hospital, University of British Columbia, Vancouver, BC, Canada.
  2. BC Children’s Hospital, University of British Columbia, Vancouver, BC, Canada.
  3. BC Children’s Hospital, University of British Columbia, Vancouver, BC, Canada.
  4. Birmingham Children’s Hospital, Birmingham, UK.
  5. Alberta Children's Hospital, University of Calgary, Calgary, AB, Canada.
  6. The University of Western Ontario, London, ON, Canada.
  7. Lucile Packard Children's Hospital Stanford, Palo Alto, CA, USA.
  8. University of California, San Francisco, CA, USA.
  9. University of California, San Francisco, CA, and Boston Children’s Hospital, Boston, MA, USA.
  10. Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, USA.
  11. NH SRCC Children's Hospital, Mumbai, India.
  12. University of Utah, Salt Lake City, UT, USA.
  13. Royal Hospital for Children, Glasgow, UK.
  14. Great North Children’s Hospital, Newcastle upon Tyne, Institute for Health and Population, Newcastle University, UK.
  15. Rigshospitalet, Copenhagen, Denmark.
  16. Division of Nephrology, Toronto SickKids Hospital, University of Toronto, ON, Canada.
  17. Aster Women & Children’s Hospital, Whitefield, Bangalore, India.
  18. Seattle Children's Hospital and Research Center, University of Washington, Seattle, WA, USA.
  19. Nationwide Children’s Hospital, Columbus, OH, USA.
  20. Montefiore Medical Center, Bronx, NY, USA.
  21. Hospital for Sick Children and University of Toronto, Toronto, ON, Canada.
  22. BC Children’s Hospital, University of British Columbia, Vancouver, BC, Canada.
  23. BC Children’s Hospital, University of British Columbia, Vancouver, BC, Canada. kmorishita@cw.bc.ca

for ARChiVe Investigators within the PedVas Initiative

CER14637
2022 Vol.40, N°4
PI 0841, PF 0848
Paediatric Rheumatology

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PMID: 35383555 [PubMed]

Received: 16/03/2021
Accepted : 14/02/2022
In Press: 01/04/2022
Published: 04/05/2022

Abstract

OBJECTIVES:
High-dose glucocorticoids for remission-induction of ANCA-associated vasculitis are recommended and commonly used in adults, but recent studies suggest lower glucocorticoid doses can reduce toxicity without reducing efficacy. No paediatric-specific data exists to inform optimal glucocorticoid dosing in paediatric ANCA-associated vasculitis (pAAV). Our objectives were to describe glucocorticoid use in pAAV-related renal disease, and to explore associations between glucocorticoid dose, baseline patient characteristics and 12-month outcomes.
METHODS:
Youth <18 years with pAAV, biopsy-confirmed pauci-immune glomerulonephritis and 12-month follow-up data were included from an international paediatric vasculitis registry. Presenting features and 12-month outcomes (eGFR, glucocorticoid-related adverse effects), were compared between patients receiving no, low-moderate (≤90mg/kg) and high (>90mg/kg) cumulative intravenous methylprednisolone (IVMP), and low (<0.5mg/kg/day prednisone equivalent), moderate (0.5-1.5mg/kg/day) and high (>1.5mg/kg/day) starting doses of oral glucocorticoids.
RESULTS:
Among 131 patients (101 granulomatosis with polyangiitis, 30 microscopic polyangiitis), 27 (21%) received no IVMP, 64 (49%) low-moderate and 29 (22%) high-dose IVMP, while 9 (7%) received low, 75 (57%) moderate and 47 (36%) high initial doses of oral glucocorticoids. Renal failure at diagnosis (p=0.022) and plasmapheresis use (p=0.0001) were associated with high-dose IVMP. Rates of glucocorticoid-related adverse effects ranged from 15-31% across dose levels, and glucocorticoid dosing did not associate with 12-month outcomes.
CONCLUSIONS:
Glucocorticoid dosing for pAAV-related renal disease was highly variable, and rates of adverse effects were high across all dosing groups. A significant proportion of patients received oral glucocorticoid or IVMP doses that were discordant with current adult guidelines. Higher glucocorticoid doses did not associate with improved outcomes.

DOI: https://doi.org/10.55563/clinexprheumatol/iol4k2

Rheumatology Article

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