Abstract
BackgroundKawasaki disease (KD) is an acute, self-limiting systemic vasculitis that predominately affects children. Neurological involvement is a known complication of KD, however, its association with KD severity remains elusive. We aimed to systematically describe the general manifestations of neurological involvement in KD, determine whether neurological involvement is a marker of disease severity in patients with KD, and assess the relationship of such involvement with intravenous immunoglobulin (IVIG) resistance and coronary artery lesions (CALs).MethodsWe retrospectively reviewed data from 1582 patients with KD between January 2013 and December 2017. Profiles of patients with neurological symptoms (group A, n = 80) were compared to those of gender- and admission date-matched patients without neurological involvement (group B, n = 512). Multivariate logistic regression analyses were performed to determine whether neurological involvement was significantly associated with IVIG resistance.ResultsNeurological involvement was observed in 5.1% (80/1582) of patients with KD. The neurological manifestations were diffuse, presenting as headache (13/80, 16.3%), convulsions (14/80, 17.5%), somnolence (40/80, 50.1%), extreme irritability (21/80, 26.3%), signs of meningeal irritation (15/80, 18.8%), bulging fontanelles (7/80, 8.8%), and facial palsy (1/80, 1.3%). Neurological symptoms represented the initial and/or predominant manifestation in 47.5% (38/80) of patients with KD. The incidence of IVIG resistance and levels of inflammatory markers were higher in group A than in group B. However, neurological involvement was not an independent risk factor for IVIG resistance or CALs.ConclusionRates of neurological involvement were relatively low in patients with KD. Neurological involvement was associated with an increased risk of IVIG resistance and severe inflammatory burden. Our results highlight the need for pediatricians to recognize KD with neurological involvement and the importance of standard IVIG therapy.Trial registrationRetrospectively registered.
Highlights
Kawasaki disease (KD) is an acute, self-limiting systemic vasculitis that predominately affects children
In patients with Cerebrospinal fluid (CSF) examination, the proportion of males was significantly higher among patients with KD with abnormal results of CSF than without; clinical characteristics and laboratory profiles showed no differences, as summarized in supplemental material 1
Our findings indicate that the incidence of neurological involvement is relatively low among patients with KD
Summary
Kawasaki disease (KD) is an acute, self-limiting systemic vasculitis that predominately affects children. We aimed to systematically describe the general manifestations of neurological involvement in KD, determine whether neurological involvement is a marker of disease severity in patients with KD, and assess the relationship of such involvement with intravenous immunoglobulin (IVIG) resistance and coronary artery lesions (CALs). KD can affect multiple organs and tissues including the pulmonary and gastrointestinal systems, thereby increasing the risk of coronary artery lesions (CALs) associated with delayed diagnosis and/or intravenous immunoglobulin (IVIG) resistance [2,3,4]. In the present study, we aimed to systematically describe the general manifestations of neurological involvement in KD, determine whether neurological involvement is a marker of disease severity in patients with KD, and assess the relationship of such involvement with IVIG resistance and CALs
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