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Kawasaki disease: a prospective population survey in the UK and Ireland from 2013 to 2015
  1. Robert M R Tulloh1,2,
  2. Richard Mayon-White3,
  3. Anthony Harnden3,
  4. Athimalaipet V Ramanan1,2,
  5. E Jane Tizard1,
  6. Delane Shingadia4,
  7. Colin A Michie5,
  8. Richard M Lynn6,
  9. Michael Levin7,
  10. Orla D Franklin8,
  11. Pippa Craggs2,
  12. Sue Davidson9,
  13. Rebecca Stirzaker10,
  14. Mike Danson10,
  15. Paul A Brogan4
  1. 1 Paediatric Cardiology, Bristol Royal Hospital for Children, Bristol, UK
  2. 2 Bristol Medical School, University of Bristol, Bristol, UK
  3. 3 Primary Care, University of Oxford, Oxford, UK
  4. 4 Rheumatology, UCL Great Ormond Street Institute of Child Health, London, UK
  5. 5 Paediatrics, Ealing General Hospital, London, UK
  6. 6 British Paediatric Surveillance Unit, Royal College of Paediatrics, London, UK
  7. 7 Paediatrics, Imperial College, London, UK
  8. 8 Paediatric Cardiology, Our Lady’s Children’s Hospital, Dublin, Ireland
  9. 9 Kawasaki Disease Support Group, Coventry, UK
  10. 10 Social Sciences, Heriot-Watt University, Edinburgh, UK
  1. Correspondence to Prof. Robert M R Tulloh, Paediatric Cardiology, Bristol Royal Hospital For Children, Bristol BS2 8BJ, UK; robert.tulloh{at}


Objective Kawasaki disease (KD) is an increasingly common vasculitis with risk of coronary artery aneurysms (CAAs). The last UK survey was in 1990, whereas current epidemiology, treatment patterns and complication rates are unknown. The aim of this study was to address this knowledge gap.

Methods A British Paediatric Surveillance Unit survey in the UK and Ireland from 1 January 2013 to 28 February 2015 ascertained demographics, ethnicity, seasonal incidence, treatment and complication rates.

Results 553 cases were notified: 389 had complete KD, 46 had atypical KD and 116 had incomplete KD; 2 were diagnosed at postmortem with an incidence of 4.55/100 000 children under 5 years, with a male to female ratio of 1.5:1 and a median age of 2.7 years (2.5 months–15 years). Presentation was highest in January and in rural areas. Most were white (64%), and Chinese and Japanese Asians were over-represented as were black African or African mixed-race children. 94% received intravenous immunoglobulin (IVIG). The overall CAA rate was 19%, and all-cardiac complications affected 28%. Those with CAA received IVIG later than in those without (median 10 days vs 7 days). Those under 1 year had fewer symptoms, but the highest CAA rate (39%). Overall 8 of 512 cases (1.6%) had giant CAA, and 4 of 86 cases (5%) under 1 year of age developed giant CAA. Mortality from KD was 0.36%.

Conclusions The UK and Ireland incidence of KD has increased and is more frequently seen in winter and rural areas. Delayed IVIG treatment is associated with CAA, suggesting earlier and adjunctive primary treatment might reduce complications to prevent CAA, particularly in the very young.

  • kawasaki disease
  • cardiology
  • epidemiology
  • paediatric practice

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  • Contributors RMRT, AH, RM-W, PAB and RML designed the project. Cases were assessed by the expert panel review (RMRT, RM-W, AVR, EJT, DS, PAB), and PC collected and recorded the data. SD provided patient participation support and informed families involved in the study of the ongoing progress. CAM, AH and ODF provided local support and assisted with recruitment. RM-W, RS and MD undertook statistical analysis.

  • Funding Kawasaki disease parent support group and discretionary institutional academic funds from each of the authors.

  • Competing interests None declared.

  • Ethics approval National Research Ethics Committee 11/SW/0310 and NHS Sponsorship CS/20113847.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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