Evaluation of Kawasaki Disease Risk-Scoring Systems for Intravenous Immunoglobulin Resistance
TL;DR: Primary steroid therapy did not improve coronary outcomes in patients prospectively classified as being at high-risk for IVIG resistance, and risk-scoring systems from Japan have good specificity but low sensitivity for predictingIVIG resistance in a North American cohort.
About: This article is published in The Journal of Pediatrics.The article was published on 2011-05-01 and is currently open access. It has received 303 citations till now. The article focuses on the topics: Framingham Risk Score & Risk factor.
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TL;DR: These recommendations provide updated and best evidence-based guidance to healthcare providers who diagnose and manage Kawasaki disease, but clinical decision making should be individualized to specific patient circumstances.
Abstract: Background: Kawasaki disease is an acute vasculitis of childhood that leads to coronary artery aneurysms in ≈25% of untreated cases. It has been reported worldwide and is the leading cause of acqui...
2,139 citations
TL;DR: Addition of prednisolone to the standard regimen of intravenous immunoglobulin improves coronary artery outcomes in patients with severe Kawasaki disease in Japan.
521 citations
TL;DR: Both coronary artery bypass surgery and percutaneous intervention have been used to treat Kawasaki disease patients who develop myocardial ischemia as a consequence of coronary artery aneurysms and stenosis.
346 citations
TL;DR: The addition of infliximab to primary treatment in acute Kawasaki disease did not reduce treatment resistance, but it was safe and well tolerated and reduced fever duration, some markers of inflammation, left anterior descending coronary artery Z score, and intravenous immunoglobulin reaction rates.
235 citations
TL;DR: Recent advances in the understanding of KD pathogenesis and therapeutics are summarized, and an approach for managing KD patients in the UK in the light of these advances is provided.
Abstract: Kawasaki disease (KD) is an acute self-limiting inflammatory disorder, associated with vasculitis, affecting predominantly medium-sized arteries, particularly the coronary arteries. In developed countries KD is the commonest cause of acquired heart disease in childhood. The aetiology of KD remains unknown, and it is currently believed that one or more as yet unidentified infectious agents induce an intense inflammatory host response in genetically susceptible individuals. Genetic studies have identified several susceptibility genes for KD and its sequelae in different ethnic populations, including FCGR2A, CD40, ITPKC, FAM167A-BLK and CASP3, as well as genes influencing response to intravenous immunoglobulin (IVIG) and aneurysm formation such as FCGR3B, and transforming growth factor (TGF) β pathway genes. IVIG and aspirin are effective therapeutically, but recent clinical trials and meta-analyses have demonstrated that the addition of corticosteroids to IVIG is beneficial for the prevention of coronary artery aneurysms (CAA) in severe cases with highest risk of IVIG resistance. Outside of Japan, however, clinical scores to predict IVIG resistance perform suboptimally. Furthermore, the evidence base does not provide clear guidance on which corticosteroid regimen is most effective. Other therapies, including anti-TNFα, could also have a role for IVIG-resistant KD. Irrespective of these caveats, it is clear that therapy that reduces inflammation in acute KD, improves outcome. This paper summarises recent advances in the understanding of KD pathogenesis and therapeutics, and provides an approach for managing KD patients in the UK in the light of these advances.
224 citations
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TL;DR: Recommendations for the initial evaluation, treatment in the acute phase, and long-term management of patients with Kawasaki disease are intended to assist physicians in understanding the range of acceptable approaches for caring for patients withawasaki disease.
Abstract: Background—Kawasaki disease is an acute self-limited vasculitis of childhood that is characterized by fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy. Coronary artery aneurysms or ectasia develop in 15% to 25% of untreated children and may lead to ischemic heart disease or sudden death. Methods and Results—A multidisciplinary committee of experts was convened to revise the American Heart Association recommendations for diagnosis, treatment, and long-term management of Kawasaki disease. The writing group proposes a new algorithm to aid clinicians in deciding which children with fever for 5 days and 4 classic criteria should undergo echocardiography, receive intravenous gamma globulin (IVIG) treatment, or both for Kawasaki disease. The writing group reviews the available data regarding the initial treatment for children with acute Kawasaki disease, as well for those who have persistent or recrudescent fever despite initial therapy with IVIG, including IVIG retreatment and treatment with corticosteroids, tumor necrosis factor- antagonists, and abciximab. Long-term management of patients with Kawasaki disease is tailored to the degree of coronary involvement; recommendations regarding antiplatelet and anticoagulant therapy, physical activity, follow-up assessment, and the appropriate diagnostic procedures to evaluate cardiac disease are classified according to risk strata. Conclusions—Recommendations for the initial evaluation, treatment in the acute phase, and long-term management of patients with Kawasaki disease are intended to assist physicians in understanding the range of acceptable approaches for caring for patients with Kawasaki disease. The ultimate decisions for case management must be made by physicians in light of the particular conditions presented by individual patients. (Circulation. 2004;110:2747-2771.)
1,854 citations
TL;DR: Predictors to predict unresponsiveness to intravenous immunoglobulin (IVIG) in Kawasaki disease (KD) showed high sensitivity and specificity in identifying IVIG nonresponders among KD patients.
Abstract: Background— In the present study, we developed models to predict unresponsiveness to intravenous immunoglobulin (IVIG) in Kawasaki disease (KD). Methods and Results— We reviewed clinical records of 546 consecutive KD patients (development dataset) and 204 subsequent KD patients (validation dataset). All received IVIG for treatment of KD. IVIG nonresponders were defined by fever persisting beyond 24 hours or recrudescent fever associated with KD symptoms after an afebrile period. A 7-variable logistic model was constructed, including day of illness at initial treatment, age in months, percentage of white blood cells representing neutrophils, platelet count, and serum aspartate aminotransferase, sodium, and C-reactive protein, which generated an area under the receiver-operating-characteristics curve of 0.84 and 0.90 for the development and validation datasets, respectively. Using both datasets, the 7 variables were used to generate a simple scoring model that gave an area under the receiver-operating-chara...
622 citations
"Evaluation of Kawasaki Disease Risk..." refers methods in this paper
...We used the dataset of the Pediatric Heart Network KDTrial cohort to: (1) assess the operating characteristics of the published Japanese risk scoring systems in a North American population; (2) examine whether risk level defined with the scoring systems is predictive of the occurrence of CA abnormalities; and (3) assess whether primary steroid therapy can be demonstrated as effective in patients who are predicted to be at high risk of IVIG resistance on the basis of factors that are known at the time of presentation....
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...01 39% (18) 46% (21) 13% (6) 2% (1) 85% (specificity) tivity) 15%...
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...0-4 Kobayashi score 0 29% 11% (1) 1 31% 33% (3) 2 11% 11% (1) 3 13% 11% (1) 4 8% 11% (1) 5 7% 22% (2) 6 2% 0....
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...0-2 Sano score 0 32% 0% 1 48% 60% (6) 2 16% 30% (4) 3 4% 10% (1) Low risk (0-1) 80% 60% High risk (2-3) 20% 40% (sensi...
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...5-3 Egami score 0 35% 17% (2) 1 33% 8% (1) 2 13% 33% (4) 3 13% 33% (4) 4 4% 8% (1) 5 3% 0% Low risk (0-2) 81% 58% High risk ($ 3) 19% 42% (sensi n 56 10 Sano score 0....
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TL;DR: Resistance to IVIG treatment can be predicted using age, illness days, platelet count, ALT, and CRP and it is found that Randomized, multicenter clinical trials are necessary to create a new strategy to treat these high-risk patients.
421 citations
"Evaluation of Kawasaki Disease Risk..." refers methods in this paper
...We used the dataset of the Pediatric Heart Network KDTrial cohort to: (1) assess the operating characteristics of the published Japanese risk scoring systems in a North American population; (2) examine whether risk level defined with the scoring systems is predictive of the occurrence of CA abnormalities; and (3) assess whether primary steroid therapy can be demonstrated as effective in patients who are predicted to be at high risk of IVIG resistance on the basis of factors that are known at the time of presentation....
[...]
...0-4 Kobayashi score 0 29% 11% (1) 1 31% 33% (3) 2 11% 11% (1) 3 13% 11% (1) 4 8% 11% (1) 5 7% 22% (2) 6 2% 0....
[...]
...0-2 Sano score 0 32% 0% 1 48% 60% (6) 2 16% 30% (4) 3 4% 10% (1) Low risk (0-1) 80% 60% High risk (2-3) 20% 40% (sensi...
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TL;DR: IVIG retreatment in patients who remain febrile after the first course of IVIG is now common, although the efficacy of this practice requires assessment with a randomized trial, as well as the prevalence of new coronary abnormalities in KD patients treated with IVIG and aspirin remains low.
Abstract: Objective.To determine the prevalence and outcome of intravenous gamma-globulin (IVIG) retreatment in patients with Kawasaki disease (KD).Method.Multicenter, retrospective survey of all children with KD evaluated at nine clinical centers across North America during a 15-month period.Results.Data wer
382 citations
TL;DR: The data do not provide support for the addition of a single pulsed dose of intravenous methylprednisolone to conventional intravenous immune globulin therapy for the routine primary treatment of children with Kawasaki disease.
Abstract: Background Treatment of acute Kawasaki disease with intravenous immune globulin and aspirin reduces the risk of coronary-artery abnormalities and systemic inflammation, but despite intravenous immune globulin therapy, coronary-artery abnormalities develop in some children. Studies have suggested that primary corticosteroid therapy might be beneficial and that adverse events are infrequent with short-term use. Methods We conducted a multicenter, randomized, double-blind, placebo-controlled trial to determine whether the addition of intravenous methylprednisolone to conventional primary therapy for Kawasaki disease reduces the risk of coronary-artery abnormalities. Patients with 10 or fewer days of fever were randomly assigned to receive intravenous methylprednisolone, 30 mg per kilogram of body weight (101 patients), or placebo (98 patients). All patients then received conventional therapy with intravenous immune globulin, 2 g per kilogram, as well as aspirin, 80 to 100 mg per kilogram per day until they w...
353 citations