A mother presents with her six-month-old, who has a two-day history of fever, vomiting, rash and poor feeding. He is miserable with coryzal symptoms, a temperature of 38.9oC, respiratory rate of 28, pulse of 180, central capillary refill of less than two seconds and a blanching maculopapular rash over the nape of the neck and trunk which is forming vesicles. He is referred to hospital. Temperature spikes continue overnight, prompting a full septic screen, before starting IV antibiotics. Urine, blood and cerebrospinal fluid culture, and rapid streptococcus testing of the throat are negative. Inflammatory markers, CRP and ESR and liver enzymes are raised. Measles, other viral testing and antistreptolysin O titre are negative. On day five of illness, a non-purulent conjunctivitis develops, but no lymphadenopathy. After a normal echocardiogram he is treated for presumed sepsis. Peeling of the skin on his fingers is seen on day 15 and atypical Kawasaki disease is suspected. He is treated with IV immunoglobulin and high-dose aspirin. A repeat echo at six weeks reveals a right coronary aneurysm.
The problem
- Kawasaki Disease is an acute, self-limiting, childhood vasculitis of unknown aetiology. Unless treated within 10 days of onset, 20% of children develop coronary artery aneurysms and are at risk of ischaemic heart disease and sudden death.
- Can occur at any age but most cases are in children under five years.1
- Most common in children of Asian ancestry.
- Can present atypically. Children – particularly those younger than one year – may have only two or three of the diagnostic criteria (see box below).
Diagnosis
- Kawasaki disease should be considered in any child with fever for five or more days.
- Fever usually precedes other features and is often unresponsive to antipyretics.
- Children with Kawasaki disease are often irritable – possibly due to aseptic meningitis.
- No diagnostic tests are available, just diagnostic criteria (see box below).
Pitfalls in diagnosis
Children may initially present with fever and an enlarged cervical lymph node. The rash and mucosal changes that follow may be mistaken for a reaction to antibiotics. Sterile pyuria may be mistaken for a partially treated UTI. Fever resolves after one to two weeks but the characteristic periungual peeling of fingers or toes, and coronary artery aneurysms, occur in the weeks after this.
Management
- Children with suspected Kawasaki disease should be urgently referred to hospital.
- Early treatment with IV immunoglobulin and high-dose aspirin (with or without steroids) significantly reduces the risk of coronary aneurysms if given within 10 days of symptom onset.
- Low-dose aspirin is continued until an echo at six weeks excludes coronary changes or indefinitely in those with echo changes.
- Long-term management, by a paediatric cardiologist, is necessary for children with cardiac sequelae.
- Avoid all live vaccines for three months following IV immunoglobulin therapy.
Dr Andrew Riordan is a consultant in paediatric infectious diseases and immunology
Dr Heather Downing is an academic clinical fellowship doctor in paediatric clinical pharmacology
Dr Gavin Cleary is a consultant paediatric rheumatologist at Alder Hey Children’s Hospital, Liverpool
Alder Hey is one of Europe’s busiest children’s hospitals providing care for over 275,000 patients each year. It has a broad range of hospital and community services for direct referral from primary care. The trust also offers more complex tertiary services – it is the designated national centre for head and face surgery and a centre of excellence for children with cancer, spinal and brain disease. Alder Hey has been chosen to be a national centre for heart surgery, a respiratory ECMO surgery centre and one of just four specialist centres to provide surgery for drug-resistant epilepsy. For more information go to alderhey.nhs.uk
Reference
1 Newburger JW, Takahashi M, Gerber MA, et al. Committee on Rheumatic Fever E, Kawasaki Disease CoCDitYAHA. Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Pediatrics 2004; 114: 1,708–33
