Kawasaki disease: a prospective population survey in the UK and Ireland from 2013 to 2015
Robert M R Tulloh1,2, Richard Mayon-White3, Anthony Harnden3
1Paediatric Cardiology, Bristol Royal Hospital for Children, Bristol, UK.
Insights
Kawasaki disease (KD) incidence has risen in the UK and Ireland, particularly in winter and rural settings. Delayed IVIG treatment correlates with coronary artery aneurysms (CAAs), highlighting the need for early intervention, especially in infants.
Area of Science:
- Pediatric Rheumatology
- Infectious Diseases
- Epidemiology
Background:
- Kawasaki disease (KD) is a leading cause of acquired heart disease in children.
- Coronary artery aneurysms (CAAs) are a significant complication of KD.
- Current epidemiological data and treatment outcomes for KD in the UK and Ireland were lacking since 1990.
Purpose of the Study:
- To determine the current epidemiology of Kawasaki disease in the UK and Ireland.
- To analyze treatment patterns and complication rates, including CAAs.
- To identify risk factors and inform clinical management strategies.
Main Methods:
- A prospective British Paediatric Surveillance Unit (BPSU) survey was conducted from January 2013 to February 2015.
- Data collected included demographics, ethnicity, seasonal and geographical incidence, treatment modalities, and complication rates.
- 553 cases of KD were notified and analyzed.
Main Results:
- The incidence of KD was 4.55/100,000 children under 5, with a male predominance (1.5:1) and median age of 2.7 years.
- Peak incidence occurred in January and in rural areas; certain ethnic groups were over-represented.
- The overall CAA rate was 19%, with higher rates in infants (<1 year) and associated with delayed IVIG treatment (median 10 days vs. 7 days).
- Mortality was 0.36%.
Conclusions:
- Kawasaki disease incidence in the UK and Ireland has increased, with a predilection for winter and rural environments.
- Delayed intravenous immunoglobulin (IVIG) treatment is linked to a higher risk of CAAs.
- Earlier and potentially adjunctive primary treatments may be crucial to prevent CAAs, especially in very young children.
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.
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