Kawasaki disease: guidelines of Italian Society of Pediatrics, part II - treatment of resistant forms and cardiovascular complications, follow-up, lifestyle and prevention of cardiovascular risks

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Abstract

This second part of practical Guidelines related to Kawasaki disease (KD) has the goal of contributing to prompt diagnosis and most appropriate treatment of KD resistant forms and cardiovascular complications, including non-pharmacologic treatments, follow-up, lifestyle and prevention of cardiovascular risks in the long-term through a set of 17 recommendations.

Guidelines, however, should not be considered a norm that limits the treatment options of pediatricians and practitioners, as treatment modalities other than those recommended may be required as a result of peculiar medical circumstances, patient’s condition, and disease severity or individual complications.

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Most cited references 85

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Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association.

J Newburger, M. Takahashi, M Gerber … (2004)

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 approximately 15% to 25% of untreated children and may lead to ischemic heart disease or sudden death. 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 > or =5 days and < or =4 classic criteria should undergo echocardiography [correction], 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-alpha 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. 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.

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Kawasaki Disease.

Jane W Newburger, Masato Takahashi, Jane C. Burns (2016)

Kawasaki disease is an acute, self-limited vasculitis of unknown etiology that occurs predominantly in infants and children. If not treated early with high-dose intravenous immunoglobulin, 1 in 5 children develop coronary artery aneurysms; this risk is reduced 5-fold if intravenous immunoglobulin is administered within 10 days of fever onset. Coronary artery aneurysms evolve dynamically over time, usually reaching a peak dimension by 6 weeks after illness onset. Almost all the morbidity and mortality occur in patients with giant aneurysms. Risk of myocardial infarction from coronary artery thrombosis is greatest in the first 2 years after illness onset. However, stenosis and occlusion progress over years. Indeed, Kawasaki disease is no longer a rare cause of acute coronary syndrome presenting in young adults. 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.

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Efficacy of immunoglobulin plus prednisolone for prevention of coronary artery abnormalities in severe Kawasaki disease (RAISE study): a randomised, open-label, blinded-endpoints trial.

Tohru Kobayashi, Tsutomu Saji, Tetsuya Otani … (2012)

Evidence indicates that corticosteroid therapy might be beneficial for the primary treatment of severe Kawasaki disease. We assessed whether addition of prednisolone to intravenous immunoglobulin with aspirin would reduce the incidence of coronary artery abnormalities in patients with severe Kawasaki disease. We did a multicentre, prospective, randomised, open-label, blinded-endpoints trial at 74 hospitals in Japan between Sept 29, 2008, and Dec 2, 2010. Patients with severe Kawasaki disease were randomly assigned by a minimisation method to receive either intravenous immunoglobulin (2 g/kg for 24 h and aspirin 30 mg/kg per day) or intravenous immunoglobulin plus prednisolone (the same intravenous immunoglobulin regimen as the intravenous immunoglobulin group plus prednisolone 2 mg/kg per day given over 15 days after concentrations of C-reactive protein normalised). Patients and treating physicians were unmasked to group allocation. The primary endpoint was incidence of coronary artery abnormalities during the study period. Analysis was by intention to treat. This trial is registered with the University Hospital Medical Information Network clinical trials registry, number UMIN000000940. We randomly assigned 125 patients to the intravenous immunoglobulin plus prednisolone group and 123 to the intravenous immunoglobulin group. Incidence of coronary artery abnormalities was significantly lower in the intravenous immunoglobulin plus prednisolone group than in the intravenous immunoglobulin group during the study period (four patients [3%] vs 28 patients [23%]; risk difference 0·20, 95% CI 0·12-0·28, p<0·0001). Serious adverse events were similar between both groups: two patients had high total cholesterol and one neutropenia in the intravenous immunoglobulin plus prednisolone group, and one had high total cholesterol and another non-occlusive thrombus in the intravenous immunoglobulin group. Addition of prednisolone to the standard regimen of intravenous immunoglobulin improves coronary artery outcomes in patients with severe Kawasaki disease in Japan. Further study of intensified primary treatment for this disease in a mixed ethnic population is warranted. Japanese Ministry of Health, Labour and Welfare. Copyright © 2012 Elsevier Ltd. All rights reserved.

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Author and article information

Contributors

Alessandra Marchesi: alessandra.marchesi@opbg.net

Isabella Tarissi de Jacobis: isabella.tarissi@opbg.net

Donato Rigante: donato.rigante@unicatt.it

Alessandro Rimini: alessandrorimini@ospedale-gaslini.ge.it

Walter Malorni: valter.malorni@iss.it

Giovanni Corsello: giocors@alice.it

Grazia Bossi: G.Bossi@smatteo.pv.it

Sabrina Buonuomo: psabrina.buonuomo@opbg.net

Fabio Cardinale: fabiocardinale@libero.it

Elisabetta Cortis: elicortis58@gmail.com

Fabrizio De Benedetti: fabrizio.debenedetti@opbg.net

Andrea De Zorzi: andrea.dezorzi@opbg.net

Marzia Duse: marzia.duse@uniroma1.it

Domenico Del Principe: domenico.delprincipe@gmail.com

Rosa Maria Dellepiane: rosamaria.dellepiane@policlinico.mi.it

Livio D’Isanto: liviodisanto@gmail.com

Maya El Hachem: may.elachem@opbg.net

Susanna Esposito: susanna.esposito@unipg.it

Fernanda Falcini: FALCINI@UNIFI.IT

Ugo Giordano: ugo.giordano@opbg.net

Maria Cristina Maggio: mariacristina.maggio@unipa.it

Savina Mannarino: savina.mannarino@smatteo.pv.it

Gianluigi Marseglia: gl.marseglia@smatteo.pv.it

Silvana Martino: silvana.martino@unito.it

Giulia Marucci: giulia.marucci@opbg.net

Rossella Massaro: rossella.massaro@yahoo.it

Christian Pescosolido: info@rarimaspeciali.net

Donatella Pietraforte: donatella.pietraforte@iss.it

Maria Cristina Pietrogrande: mariacristina.pietrogrande@unimi.it

Patrizia Salice: patriziasalice@gmail.com

Aurelio Secinaro: aurelio.secinaro@opbg.net

Elisabetta Straface: elisabetta.straface@iss.it

Alberto Villani: alberto.villani@opbg.net

Journal

Journal ID (nlm-ta): Ital J Pediatr

Journal ID (iso-abbrev): Ital J Pediatr

Title: Italian Journal of Pediatrics

Publisher: BioMed Central (London )

ISSN (Electronic): 1824-7288

Publication date (Electronic): 30 August 2018

Publication date PMC-release: 30 August 2018

Publication date Collection: 2018

Volume: 44

Electronic Location Identifier: 103

Affiliations

[1 ]ISNI 0000 0001 0727 6809, GRID grid.414125.7, Bambino Gesù Children’s Hospital, ; Piazza S. Onofrio n. 4, 00165 Rome, Italy

[2 ]ISNI 0000 0001 0941 3192, GRID grid.8142.f, Università Cattolica Sacro Cuore, Fondazione Policlinico A. Gemelli IRCCS, ; Rome, Italy

[3 ]ISNI 0000 0004 1760 0109, GRID grid.419504.d, Giannina Gaslini Institute, ; Genoa, Italy

[4 ]ISNI 0000 0000 9120 6856, GRID grid.416651.1, Istituto Superiore di Sanità, ; Rome, Italy

[5 ]ISNI 0000 0004 1762 5517, GRID grid.10776.37, Università degli Studi di Palermo, ; Palermo, Italy

[6 ]ISNI 0000 0004 1762 5736, GRID grid.8982.b, Università degli Studi di Pavia, ; Pavia, Italy

[7 ]Policlinico Giovanni XXIII, Bari, Italy

[8 ]ISNI 0000 0004 1760 4441, GRID grid.416628.f, Ospedale S. Eugenio, ; Rome, Italy

[9 ]GRID grid.7841.a, Università degli Studi Sapienza, ; Rome, Italy

[10 ]ISNI 0000 0001 2300 0941, GRID grid.6530.0, Università degli Studi Tor Vergata, ; Rome, Italy

[11 ]ISNI 0000 0004 1757 8749, GRID grid.414818.0, Ospedale Maggiore Policlinico, ; Milan, Italy

[12 ]Ospedale di Battipaglia, Salerno, Italy

[13 ]ISNI 0000 0004 1757 3630, GRID grid.9027.c, Università degli Studi di Perugia, ; Perugia, Italy

[14 ]ISNI 0000 0004 1757 2304, GRID grid.8404.8, University of Florence, ; Florence, Italy

[15 ]ISNI 0000 0001 2336 6580, GRID grid.7605.4, Università degli Studi di Torino, ; Turin, Italy

[16 ]Associazione Rari ma Speciali, Rome, Italy

Article

Publisher ID: 529

DOI: 10.1186/s13052-018-0529-2

PMC ID: 6116479

PubMed ID: 30157893

SO-VID: 5cf747b0-646c-4eb9-ae0a-c353147ea39c

License:

Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

History

Date received : 4 January 2018

Date accepted : 29 July 2018

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ScienceOpen disciplines: Pediatrics

Keywords: kawasaki disease,coronary artery abnormalities,intravenous immunoglobulin,aspirin,personalized medicine,innovative biotechnologies,child

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ScienceOpen disciplines: Pediatrics

Keywords: kawasaki disease, coronary artery abnormalities, intravenous immunoglobulin, aspirin, personalized medicine, innovative biotechnologies, child

Kawasaki disease: guidelines of Italian Society of Pediatrics, part II - treatment of resistant forms and cardiovascular complications, follow-up, lifestyle and prevention of cardiovascular risks

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Pediatric Pulse: Advancing Neonatal and Child Health

Most cited references 85

Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association.

Kawasaki Disease.

Efficacy of immunoglobulin plus prednisolone for prevention of coronary artery abnormalities in severe Kawasaki disease (RAISE study): a randomised, open-label, blinded-endpoints trial.

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