Targeted use of prednisolone with the second <scp>IVIG</scp> dose for refractory <scp>K</scp>awasaki disease

Kimura, Mitsuaki; Harazaki, Masashi; Fukuoka, Tomoki; Asakura, Isao; Sakai, Harumi; Kamimaki, Tsutomu; Ohkawara, Ichiro; Akiyama, Nobutake; Tsurui, S; Iwashima, Satoru; Shimomura, Masaki; Morishita, Hideaki; Meguro, Takaaki; Seto, Shiro

doi:10.1111/ped.13190

Cited by 17 publications

(6 citation statements)

References 26 publications

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“…Early use of corticosteroids during the acute phase appears to be more beneficial than in refractory (IVIG-resistant) cases [ 67 , 73 ]. Patients at higher risk of poor coronary outcomes have the greatest magnitude of benefit from early adjunctive corticosteroid therapy [ 68 , 69 , 72 , 74 ]. Overall results demonstrate good tolerability and safety with corticosteroid use and no evidence of increased incidence of adverse outcomes.…”

Section: Introductionmentioning

confidence: 99%

Kawasaki Disease: an Update

2020

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Purpose of Review Provide the most recent updates on the epidemiology, pathogenesis, and treatment advances in Kawasaki disease. Recent Findings Treatment advances in complex, IVIG-refractory cases of Kawasaki disease. Multisystem inflammatory syndrome, a newly reported inflammatory condition with Kawasaki-like features and an association with the 2019 Coronavirus (COVID-19). Summary Kawasaki disease (KD) is a rare systemic inflammatory disease that predominately affects children less than 5 years of age. Pathogenesis of KD remains unknown; the leading theory is that an unknown stimulus triggers an immune-mediated inflammatory cascade in a genetically susceptible child. Classic KD is a clinical diagnosis based on set criteria and excluding other similar clinical entities. Patients who do not fulfill complete diagnostic criteria for KD are often referred to as atypical (or incomplete) KD. The most feared complication of KD is coronary artery abnormality development, and patients with atypical KD are also at risk. Administration of intravenous immunoglobulin (IVIG) and aspirin has greatly reduced the incidence of coronary lesions in affected children. Several other immune-modulating therapies have recently been utilized in complex or refractory cases. Keywords Kawasaki disease. Kawasaki review. Kawasaki treatment. Kawasaki workup. Kawasaki differential. Kawasaki diagnosis. Multi system inflammatory syndrome. Kawasaki-like disease Pathogenesis Genetics Although poorly understood, the predilection for children of East Asian and Pacific Islander descent, even with transmigration, This article is part of the Topical Collection on Vasculitis * Eileen Rife

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Section: Introductionmentioning

confidence: 99%

Kawasaki Disease: an Update

2020

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“…An intensified initial rescue therapy may reduce the occurrence of repeated IVIG resistance, but whether apply to every resistant KD child is controversial when taking into account potential adverse outcomes and the economic cost of the treatment [8][9][10]. It seems more reasonable to consider intensified therapy if KD children with repeated IVIG resistance,that have high risk of coronary artery aneurysm (CAA) [11]. Therefore, early identification of repeated IVIG resistance could help physicians make smarter therapeutic decisions to reduce CAA and medical costs.…”

Section: Introductionmentioning

confidence: 99%

Prediction of repeated intravenous immunoglobulin resistance in children with Kawasaki disease

et al. 2021

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Background Repeated intravenous immunoglobulin (IVIG) resistance prediction is one of the pivotal topics in Kawasaki disease (KD). Those non-responders of repeated IVIG treatment might be improved by an early-intensified therapy to reduce coronary artery lesion and medical costs. This study investigated predictors of resistance to repeated IVIG treatment in KD. Methods A total of 94 children with IVIG-resistant KD treated at our hospital between January 2016 and August 2020 were retrospectively analyzed. According to the therapeutic effect of a second dose IVIG treatment, the children were divided into repeated IVIG-responsive group and repeated IVIG-resistant group, and the clinical and laboratory data were compared. Predictors of repeated IVIG resistance and the optimal cut-off value were determined by multiple logistic regression analysis and receiver operating characteristic (ROC) curve analysis. Results The Pre-IVIG laboratory data showed the percentage of neutrophils (N%) and levels of serum procalcitonin (PCT), N-terminal pro-brain natriuretic peptide (NT-proBNP) were significantly higher in repeated IVIG-resistant group compared with repeated IVIG-responsive group, while levels of serum sodium and albumin (ALB) were significantly lower (P < 0.05). The post-IVIG laboratory values of N% and C-reactive protein (CRP) were significantly higher in the repeated IVIG-resistant group compared with repeated IVIG-responsive group, while hemoglobin and ALB were lower (P < 0.05). Pre-IVIG PCT and post-IVIG CRP exhibited AUC of 0.751 and 0.778 respectively in predicting repeated IVIG resistance in KD. Pre-IVIG PCT > 1.81ng/ml (OR 4.1, 95 % CI 1.4 ~ 12.0, P < 0.05) and post-IVIG CRP > 45 mg/L (OR 4.6, 95 % CI 1.3 ~ 16.2, P < 0.05) were independent predictors of repeated IVIG resistance in KD. Conclusions Our study illustrates the serum PCT level before initial IVIG treatment and CRP after initial IVIG could be used to predict repeated IVIG resistance in KD.

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“…The adjunction of steroids to the second IVIG dose seems to be an effective second treatment line but the optimal treatment for refractory KD remains controversial (14).…”

Section: Discussionmentioning

confidence: 99%

Resolution of Giant Coronary Aneurisms in a Child With Refractory Kawasaki Disease Treated With Anakinra

et al. 2020

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Kawasaki disease (KD) is an acute, febrile illness of unknown etiology that mainly affects children under 5 years of age. intravenous immunoglobulin (IVIG), the standard treatment, has reduced coronary involvement to <5%. Patients who do not improve after an initial IVIG have a higher risk of developing coronary arteries aneurysms, and its optimal treatment remains controversial. We present a case of IVIG, steroids, and infliximab-resistant KD in a 9-month-old child, which developed giant aneurysms and was successfully treated with anakinra, a recombinant antagonist of the IL-1 receptor. In our case, the introduction of IL-1 receptor antagonist therapy seems to have blocked the disease from both a clinical and a laboratory point of view. We also noted a very rapid regression of coronary aneurysms passed from giant aneurysms to small ones, or, as in the case of the anterior descending artery, the complete disappearance of the aneurysm formation. We think that our case adds more evidences to the potential role of IL-1RA as therapy in some selected cases of refractory KD, in particular with severe involvement of coronary arteries, although new efficacy trials are needed to better understand the role of Anakinra in these patients.

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Targeted use of prednisolone with the second IVIG dose for refractory Kawasaki disease

Cited by 17 publications

References 26 publications

Kawasaki Disease: an Update

Kawasaki Disease: an Update

Prediction of repeated intravenous immunoglobulin resistance in children with Kawasaki disease

Resolution of Giant Coronary Aneurisms in a Child With Refractory Kawasaki Disease Treated With Anakinra

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