Specific Conditions |
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Indication for Ig Use |
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Level of Evidence | Clear evidence of benefit (Category 1) |
Description and Diagnostic Criteria |
Kawasaki disease is an acute, febrile, multi-system disease of children and young infants often involving the coronary arteries. Coronary artery dilatation, which may result in aneurysm formation, may be noted at presentation and most commonly occurs in the sub-acute phase of the illness, from day 10 onwards (onset of fever is day one). The cause of the condition is unknown, but there is evidence that the characteristic vasculitis results from an exaggerated pro-inflammatory immunological response that involves both the innate and adaptive arms of the immune system, characterised by T-cell and macrophage activation to an unknown antigen, secretion of cytokines, polyclonal B-cell hyperactivity. It is likely that in genetically susceptible individuals, one or more uncharacterised common infectious agents, possibly with super-antigen activity, may trigger the disease. Diagnosis A diagnosis of Kawasaki disease is generally made if fever of four or more days’ duration is associated with at least four of the following cardinal diagnostic criteria, which often appear sequentially, or three if coronary abnormalities are evident on echocardiogram:
Data support the use of intravenous immunoglobulin (IVIg) while there is ongoing inflammation (usually taken as ongoing fever or raised acute inflammatory markers). Prognosis is worse if IVIg is used 10 days post-onset, but should be used at any time if there is evidence of inflammation. Up to 15 percent of patients do not respond to initial IVIg therapy. Consensus is for re-treatment with 2 g/kg of IVIg before considering steroids. |
Justification for Evidence Category | One high-quality systematic review of 16 randomised controlled trials (RCTs) that showed that IVIg is of benefit in treating Kawasaki disease (Oates-Whitehead et al 2003). |
Diagnosis Requirements |
A diagnosis must be made by an Immunologist, Rheumatologist, Paediatrician, Intensivist, Paediatric Infectious Diseases Physician or a Cardiologist. |
Qualifying Criteria for Ig Therapy |
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Review Criteria for Assessing the Effectiveness of Ig Use |
Review is not mandated for this indication however the following criteria may be useful in assessing the effectiveness of therapy.
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Dose |
The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.
Refer to the current product information sheet for further information on dose, administration and contraindication. |
Bibliography |
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Biotext 2004, ‘Summary data on conditions and papers’, in A systematic literature review and report on the efficacy of intravenous immunoglobulin therapy and its risks, commissioned by the National Blood Authority on behalf of all Australian Governments, pp. 255-6. Available from: https://www.blood.gov.au/system/files/A-systematic-literature-review-and-report-on-the-efficacy-of-IVIg-therapy-and-its-risks.pdf Burns, JC, & Glode, MP, 2004, ‘Kawasaki syndrome’, Lancet, vol. 364, no. 9433, pp. 533–44. https://www.ncbi.nlm.nih.gov/pubmed/15302199 De Zorzi, A, Colan, SD, Gauvreau, K, et al 1998, ‘Coronary artery dimensions may be misclassified as normal in Kawasaki disease’, Journal of Pediatrics, vol. 133, no. 2, pp. 254–8. https://www.ncbi.nlm.nih.gov/pubmed/9709715 Durongpisitkul, K, Soongswang, J, Laohaprasitiporn, D, et al 2003, ‘Immunoglobulin failure and retreatment in Kawasaki disease’, Paediatric Cardiology, vol. 24, no. 2, pp. 145–8. http://link.springer.com/article/10.1007/s00246-002-0216-2 Feigin, RD, Cecchin, F, & Wissman, SD, 2006, ‘Kawasaki disease’, in JA McMillan (ed.), Oski’s paediatrics: principles and practice, 4th edn, Lippincott Williams & Wilkins, Philadelphia, pp. 1015–20. Harwood R et al 2020, ‘A national consensus management pathway for paediatric inflammatory multisystem syndrome temporally associated with COVID-19 (PIMS-TS): results of a national Delphi process’, The Lancet, https://doi.org/10.1016/S2352-4642(20)30304-7 Li, Z., Cai, J., Lu, J., Wang, M., Yang, C., Zeng, Z., Tang, Q., Li, J., Tang, W., Luo, H. and Pan, G., 2023. The therapeutic window of intravenous immunoglobulin (IVIG) and its correlation with clinical outcomes in Kawasaki disease: a systematic review and meta-analysis. Italian Journal of Pediatrics, 49(1), pp.1-13. Lin, YJ, Cheng, MC, Lo, MH & Chien, SJ 2015, ‘Early Differentiation of Kawasaki Disease Shock Syndrome and Toxic Shock Syndrome in a Pediatric Intensive Care Unit’, The Pediatric Infectious Disease Journal, vol. 34, no. 11, pp. 1163-7. https://www.ncbi.nlm.nih.gov/pubmed/26222065 Newburger,JW, Takahashi, M, Gerber, MA, et al 2004, ‘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’, Paediatrics, vol. 114, no. 6, pp.1708–33. https://www.ncbi.nlm.nih.gov/pubmed/15574639 Oates-Whitehead, RM, Baumer, JH, Haines, L, et al 2003, ‘Intravenous immunoglobulin for the treatment of Kawasaki disease in children (Cochrane Review)’, in The Cochrane Library, Issue 4, John Wiley & Sons, Ltd, Chichester, United Kingdom. https://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0012467/ Rosenfeld, EA, Shulman, ST, Corydon,KE, et al 1995, ‘Comparative safety and efficacy of two immune globulin products in Kawasaki disease’, Journal of Paediatrics, vol. 126, no. 6, pp.1000–1003. http://europepmc.org/abstract/med/7776074 Stiehm, ER 2006, ‘Lessons from Kawasaki disease: all brands of IVIg are not equal’, Journal of Paediatrics, vol. 148, Issue 1, pp.7–8. http://www.jpeds.com/article/S0022-3476(05)00901-7/abstract Tsai, MH, Huang, YC, Yen, MH, et al 2006, ‘Clinical responses of patients with Kawasaki disease to different brands of intravenous immunoglobulin’, Journal of Paediatrics, vol. 148, no. 1, pp.38–43. https://www.researchgate.net/publication/7348369_Clinical_Responses_of_Patients_with_Kawasaki_Disease_to_Different_Brands_of_Intravenous_Immunoglobulin Wang, CL, Wu, YT, Liu, CA, et al 2005, ‘Kawasaki disease: infection, immunity and genetics’, The Pediatric Infectious Disease Journal, vol. 24, no. 11, pp. 998–1004. https://www.ncbi.nlm.nih.gov/pubmed/16282937 |