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Rheumatology Advance Access originally published online on June 17, 2009
Rheumatology 2009 48(8):978-986; doi:10.1093/rheumatology/kep148
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© The Author 2009. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Biologic therapy in primary systemic vasculitis of the young

Despina Eleftheriou1, Marianna Melo1, Stephen D. Marks2, Kjell Tullus2, John Sills3, Gavin Cleary3, Pavla Dolezalova4, Seza Ozen5, Clarissa Pilkington1, Pat Woo1, Nigel Klein6, Michael J. Dillon2 and Paul A. Brogan1

1Rheumatology Department, 2Nephrourology Unit, Institute of Child Health, London, 3Rheumatology Department, Alder Hey Children's NHS Foundation Trust, Liverpool, UK, 4Department of Paediatrics and Adolescent Medicine, 1st Faculty of Medicine, Charles University in Prague, Prague, Czech Republic, 5Department of Paediatrics, Hacettepe University, Ankara, Turkey and 6Infectious Diseases and Microbiology Unit, Institute of Child Health, London, UK.

Correspondence to: Despina Eleftheriou, Rheumatology Department, Institute of Child Health, 30 Guilford Street, London, WC1N1EH, UK. E-mail: d.eleftheriou{at}ich.ucl.ac.uk


  Abstract

Objectives. To describe the biologic treatment regimens and report the efficacy and safety of biologic therapies in a multicentre series of children with primary systemic vasculitis (PSV).

Methods. This was a retrospective descriptive case series of children with PSV treated with biologic therapy between February 2002 and November 2007. Primary retrospective outcome assessment measures were: daily corticosteroid dose; Birmingham Vasculitis Activity Score (BVAS); and adverse events (including infection rate).

Results. Twenty-five patients median age 8.8 (range 2.4–16) years; 11 male with active PSV (n = 6 with anti-neutrophil cytoplasmic antibody associated vasculitides, n = 11 with polyarteritis nodosa, n = 7 with unclassified vasculitis and n = 1 with Behçet's disease) were treated with biologic agents including infliximab (n = 7), rituximab (n = 6), etanercept (n = 4), adalimumab (n = 1) or multiple biologics sequentially (n = 7). Overall, there was a significant reduction in BVAS from a median of 8.5 (range 5–32) at start of therapy to 4 (range 0–19) at median 32 months follow-up (P = 0.003) accompanied by significant reduction in median daily prednisolone requirement from 1 (range 0.2–2) to 0.25 (range 0–1) mg/kg/day, P = 0.000. For those receiving multiple biologic agents sequentially, a similar clinical improvement was observed with corticosteroid sparing. Infections occurred in 24%, the most severe in those receiving infliximab.

Conclusion. Our data provide retrospective evidence of efficacy of these agents, and highlight the associated infectious complications. Further multicentre standardization of treatment protocols and data collection to inform clinical trials of biologic therapy in systemic vasculitis of the young is required.

KEY WORDS: Primary systemic vasculitis, Children, Biologic therapies, Birmingham Vasculitis Activity Score, Infection rate

Submitted 30 October 2008; revised version accepted 12 May 2009.
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