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Thinking beyond intravenous immunoglobulin for Kawasaki disease
  1. Anne Sage1,
  2. Ankur Kumar Jindal2,
  3. Athimalaipet V Ramanan1
  1. 1 Department of Paediatric Rheumatology, Bristol Royal Hospital for Children, Bristol, Bristol, UK
  2. 2 Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India
  1. Correspondence to Athimalaipet V Ramanan, Department of Paediatric Rheumatology, Bristol Royal Hospital for Children, Bristol, UK; a.ramanan{at}bristol.ac.uk

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Kawasaki disease (KD), a medium vessel vasculitis of unknown aetiology, remains the leading cause of acquired heart disease in children of developed nations and is being increasingly recognised in low-income and middle-income countries. Although introduction of intravenous immunoglobulin (IVIG) has been unequivocally proven to significantly reduce the risk of KD-associated coronary artery aneurysms (CAAs), the literature suggests that as high as 19% of children will still develop cardiac sequelae even with timely administration.1 Established risk factors for development of CAAs include coronary artery dilatation at baseline echocardiogram, young age (<12 months), male sex, persistence or recrudescence of fever 36 hours post IVIG administration and Asian race.2 Given the acute and long-term morbidity that can be associated with coronary artery involvement, several adjunctive agents to IVIG have been studied for primary intensification or subsequent treatment. Despite extensive research, heterogeneity of study subjects, drug dosing, outcome measures and follow-up limits the strength of evidence to date, although additional anti-inflammatory treatment at presentation for those with established CAAs appears to improve outcomes.

Infliximab, a tumour necrosis factor-alpha (TNF-α) monoclonal antibody, is one agent that has been studied both as adjunctive first-line or subsequent treatment, with introduction of the agent as standard practice in local protocols for a decade. Tremoulet et al,3 conducted a randomised controlled trial of 5 mg/kg of …

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Footnotes

  • Contributors AVR and AS conceptualised the draft. AS and AJ drafted the manuscript. AS, AJ and AVR critically revised the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Commissioned; internally peer reviewed.

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