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

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.

• Authors: Audrea Chen, Cherry Mammen, Jaime Guzman, Eslam Al-Abadi, Susanne M Benseler, Roberta A Berard, Dana Gerstbacher, Merav Heshin-Bekenstein, Susan Kim, Marisa Klein-Gitelman, Pallavi Pimpale Chavan, Karen E James, Neil Martin, Flora McErlane, Charlotte Myrup, Damien G Noone, Jyothi Raghuram, Susan Shenoi, Vidya Sivaraman, Tamara Tanner, Rae S M Yeung, David A Cabral, Kimberly A Morishita, for ARChiVe Investigators within the PedVas Initiative
• Journal: Clinical and experimental rheumatology (Clin Exp Rheumatol) Vol. 40 Issue 4 Pg. 841-848 (May 2022) ISSN: 0392-856X [Print] Italy
• PMID: 35383555 (Publication Type: Journal Article)

Chemical References

• Antibodies, Antineutrophil Cytoplasmic
• Glucocorticoids
• Immunosuppressive Agents
• Rituximab

Topics

• Adolescent
• Adult
• Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
• Antibodies, Antineutrophil Cytoplasmic
• Child
• Female
• Glucocorticoids (therapeutic use)
• Humans
• Immunosuppressive Agents (therapeutic use)
• Male
• Microscopic Polyangiitis
• Remission Induction
• Rituximab (therapeutic use)