Abstract
BackgroundKawasaki disease (KD) is a febrile childhood vasculitis of unknown etiology. The diagnosis is highly concerning as over a quarter of children who fail to receive timely treatment with intravenous immunoglobulin (IVIG) will develop coronary aneurysms. Diagnosis relies on proper symptomatology and is supported by non-specific markers of inflammation. Previous studies have identified elevated plasma levels of interleukin-21 (IL-21) as a sensitive and specific biomarker in KD. The aim of this study is to assess the validity of IL-21 as a diagnostic biomarker for KD in febrile children in North America.MethodsPlasma samples were collected from children who presented to an urban Emergency Department in North America. IL-21 levels were measured using commercial ELISA kits in 12 KD versus 60 controls subjects.ResultsOur study shows that IL-21 levels were non-specifically elevated across all febrile children, irrespective of KD diagnosis. Length of fever prior to sample collection does not correlate with IL-21 levels. Other inflammatory markers and laboratory values were also compared to IL-21 and show no significant correlation.ConclusionsSince IL-21 is elevated non-specifically in this cohort, our data supports that IL-21 is not an appropriate biomarker for diagnosis of KD in North American pediatric populations.
Highlights
Kawasaki disease (KD) is a febrile childhood vasculitis of unknown etiology
Subjects consisted of children aged nine months to six years of age who presented to the Emergency Department of Women and Children’s Hospital of Buffalo (WCHOB) between March 2014 and March 2015
White blood cell (WBC) count was elevated in KD subjects, but this did not show statistical significance (14.7 vs 10.7 × 106/mL)
Summary
Kawasaki disease (KD) is a febrile childhood vasculitis of unknown etiology. The diagnosis is highly concerning as over a quarter of children who fail to receive timely treatment with intravenous immunoglobulin (IVIG) will develop coronary aneurysms. The classic KD presentation is a systemic vasculitis with minimum of 5 days of fever and 4 of the 5 following clinical criteria; conjunctivitis, rash, distal extremity swelling, oral mucous membrane inflammation, and non-generalized lymphadenopathy [3] Both incomplete forms, presenting with less than 4 criteria, and atypical variants, presenting classically but with an additional clinical finding not typically seen with KD, have a similar coronary arteritis propensity to classic KD presentations [4]. IgA+ plasma cells are shown within inflammatory infiltrates in the vessel walls of aneurysms from KD children [10, 11], and depleted in the peripheral blood compartment [12] These IgA+ cell rich aneurysmal infiltrates are proposed to be specific responses to an infiltrating pathogen
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