Paediatric Rheumatology
Wide variation in glucocorticoid dosing in paediatric ANCA-associated vasculitis with renal disease: a paediatric vasculitis initiative study
A. Chen1, C. Mammen2, J. Guzman3, E. Al-Abadi4, S.M. Benseler5, R.A. Berard6, D. Gerstbacher7, M. Heshin-Bekenstein8, S. Kim9, M. Klein-Gitelman10, P.P. Chavan11, K.E. James12, N. Martin13, F. Mcerlane14, C. Myrup15, D.G. Noone16, J. Raghuram17, S. Shenoi18, V. Sivaraman19, T. Tanner20, R.S. Yeung21, D.A. Cabral22, K.A. Morishita23
- BC Children's Hospital, University of British Columbia, Vancouver, BC, Canada.
- BC Children’s Hospital, University of British Columbia, Vancouver, BC, Canada.
- BC Children’s Hospital, University of British Columbia, Vancouver, BC, Canada.
- Birmingham Children’s Hospital, Birmingham, UK.
- Alberta Children's Hospital, University of Calgary, Calgary, AB, Canada.
- The University of Western Ontario, London, ON, Canada.
- Lucile Packard Children's Hospital Stanford, Palo Alto, CA, USA.
- University of California, San Francisco, CA, USA.
- University of California, San Francisco, CA, and Boston Children’s Hospital, Boston, MA, USA.
- Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, USA.
- NH SRCC Children's Hospital, Mumbai, India.
- University of Utah, Salt Lake City, UT, USA.
- Royal Hospital for Children, Glasgow, UK.
- Great North Children’s Hospital, Newcastle upon Tyne, Institute for Health and Population, Newcastle University, UK.
- Rigshospitalet, Copenhagen, Denmark.
- Division of Nephrology, Toronto SickKids Hospital, University of Toronto, ON, Canada.
- Aster Women & Children’s Hospital, Whitefield, Bangalore, India.
- Seattle Children's Hospital and Research Center, University of Washington, Seattle, WA, USA.
- Nationwide Children’s Hospital, Columbus, OH, USA.
- Montefiore Medical Center, Bronx, NY, USA.
- Hospital for Sick Children and University of Toronto, Toronto, ON, Canada.
- BC Children’s Hospital, University of British Columbia, Vancouver, BC, Canada.
- 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.