ePrints

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

Lookup NU author(s): Dr Flora McErlane

Downloads

Full text for this publication is not currently held within this repository. Alternative links are provided below where available.

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.

Publication metadata

Author(s): Chen A, Mammen C, Guzman J, Al-Abadi E, Benseler SM, Berard RA, Gerstbacher D, Heshin-Bekenstein M, Kim S, Klein-Gitelman M, Chavan PP, James KE, Martin N, McErlane F, Myrup C, Noone DG, Raghuram J, Shenoi S, Sivaraman V, Tanner T, Yeung RSM, Cabral DA, Morishita KA

Publication type: Article

Publication status: Published

Journal: Clinical and Experimental Rheumatology

Year: 2022

Volume: 40

Issue: 4

Pages: 841-848

Online publication date: 04/05/2022

Acceptance date: 14/02/2022

ISSN (print): 0392-856X

ISSN (electronic): 1593-098X

Publisher: Clinical and Experimental Rheumatology

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

DOI: 10.55563/clinexprheumatol/iol4k2

PubMed id: 35383555

Altmetrics

Share