Kawasaki disease

Kawasaki disease is the second most common vasculitis in childhood (the most common being IgA vasculitis).

Presents with an acute self-limiting febrile illness usually in young children, and is the leading cause of acquired heart disease in children aged under 5 years in the developed world.

Classic features include fever, rash, conjunctivitis, lymphadenopathy, changes to the lips or oral mucosal, and peripheries, with initial swelling and erythema followed by desquamation in the second week.

The most serious sequelae is coronary artery aneurysm, affecting 20% to 25% of untreated patients and significant proportions of treated patients in some countries, with even higher coronary artery aneurysm complication rates in those aged under 1 year.

Current standard treatment includes intravenous immunoglobulin (IVIG), alongside aspirin. Adjunctive anti-inflammatory therapy (corticosteroids or tumour necrosis factor [TNF]-alpha inhibitors) should be considered early as part of initial therapy for high-risk patients and those with Kawasaki shock syndrome.

In IVIG-resistant cases, treatments which may be of benefit include corticosteroids, TNF-alpha inhibitors, ciclosporin, anakinra, or cyclophosphamide.

Kawasaki disease (KD) is an acute, febrile, self-limiting, systemic vasculitis, the exact aetiology of which is not fully understood that almost exclusively affects young children. In an immunogenetically pre-disposed host, a yet unknown trigger enters via the airway and results in an aberrant inflammatory host response leading to the clinical manifestations. Clinically, it is characterised by fever, polymorphic rash, conjunctivitis, mucosal erythema with strawberry tongue, erythema and swelling of the hands and feet, and unilateral cervical lymphadenopathy. The most serious complication is the development of coronary artery aneurysms, which account for significant morbidity and mortality and affect 20% to 25% of untreated patients. KD is the leading cause of acquired heart disease in children aged under 5 years in the US and other developed countries.