Treatment Options for Resistant Kawasaki Disease

Phuong, Linny Kimly; Curtis, Nigel; Gowdie, Peter; Akikusa, Jonathan; Burgner, David

doi:10.1007/s40272-017-0269-6

Cited by 21 publications

(25 citation statements)

References 154 publications

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“…Notwithstanding, at least 5% of children remain febrile despite multiple dose of IVIG [18]. These particular patients are at even greater risk of CA complications, and additional therapies are usually administered [19], including corticosteroids [20][21][22], anti-TNF alpha agents [23,24], cyclosporine [25], cyclophosphamide [26] and more recently anakinra (anti-interleukine1) [27,28]. The use of these additional therapies is based on effectiveness in other vasculitis, with no prospective clinical trials to show effectiveness on coronary artery outcome.…”

Section: Discussionmentioning

confidence: 99%

Profile of resistance to IVIG treatment in patients with Kawasaki disease and concomitant infection

Dionne

Poupart

et al. 2018

PLoS ONE

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IntroductionKawasaki disease (KD) can be associated with concomitant viral or bacterial infections. Children with persistent or recurrent fever 36 hours after the end of intravenous immunoglobulin (IVIG) are considered to be resistant to treatment and are at increased risk for coronary complications. Although concomitant infection does not affect coronary outcome, it is unknown how it influences the response to IVIG treatment.MethodologyRetrospective cohort study between 2008 and 2016 in a tertiary pediatric university hospital, including 154 children, of which 59 (38%) had concomitant infection.ResultsChildren with concomitant infection were more likely to have fever 48 hours after initial IVIG treatment (36% vs 20%, p = 0.05) and to be treated with a second dose (33% vs 18%, p = 0.04). Children with infection had higher C-reactive protein at the time of diagnosis (148 vs 112 mg/L, p = 0.04), and 48 hours after IVIG administration (111 vs 59 mg/L, p = 0.003). Nevertheless, there was no statistically significant difference in the prevalence of coronary complications (Z-score > 2.5) between children with and without concomitant infection (36% vs 39%, p = 0.68).ConclusionChildren with KD and concomitant infection are more likely to have persistent fever and elevated inflammatory markers after treatment. This association increases the likelihood of receiving a second dose of IVIG but not the risk of coronary complication. Accordingly, prospective studies to distinguish true IVIG resistance from infection induced persistent fever is warranted.

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Section: Discussionmentioning

confidence: 99%

Profile of resistance to IVIG treatment in patients with Kawasaki disease and concomitant infection

Dionne

Poupart

et al. 2018

PLoS ONE

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“…1). 3,7,11 Further IVIG and/or corticosteroids are widely used in these patients. Biological agents include anti-tumour necrosis factor (infliximab, etanercept) and antiinterleukin-1 (anakinra, canakinumab).…”

Section: A Viral Infection Makes Kd Unlikely: Incorrectmentioning

confidence: 99%

“…While anakinra has shown some promise, experience is limited to case-series. 11 All children should receive high-dose aspirin in addition to IVIG: Incorrect While aspirin (ASA) has been almost universally used in treatment of KD since its first description, it has not been shown in randomised control trials to prevent CAA at any dose. 12 Some centres give high-dose ASA for anti-inflammatory effect during the acute illness, at doses ranging from 30 to 100 mg/kg/day and for variable duration.…”

Section: A Viral Infection Makes Kd Unlikely: Incorrectmentioning

confidence: 99%

Kawasaki disease fact check: Myths, misconceptions and mysteries

Butters

Curtis

Burgner

2020

J Paediatrics Child Health

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Kawasaki disease (KD) is an important cause of childhood vasculitis and a common cause of acquired heart disease in children world‐wide. The emergence of Paediatric Multisystem Inflammatory Syndrome‐Temporally Associated with SARS‐CoV‐2, a KD‐like hyperinflammatory syndrome and the recent death of Dr Tomisaku Kawasaki make this a timely review. Although KD was described by Dr Kawasaki over 50 years ago, there is still no specific diagnostic test and the aetiology remains elusive. This article summarises the latest evidence, highlights important myths and misconceptions and discusses some of the mysteries that surround this disease.

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“…Between 10% and 20% of patients have IVIG-resistant KD. Interpretation of studies investigating management of resistant KD is difficult as there is considerable variation in study populations, definitions of resistance, initial empirical management and exclusion of patients with coronary artery lesions at baseline 10. The AHA guideline recommends a second dose of IVIG or alternatively high-dose pulsed corticosteroids or infliximab 2…”

Section: Critical Appraisalmentioning

confidence: 99%