Main Article Content

Donato Rigante http://orcid.org/0000-0001-7032-7779


Kawasaki disease


Although etiology of Kawasaki disease remains elusive, the available evidence indicates that the primum movens might be a dysregulation of immune responses to various microbes, i.e. a kind of immune-mediated response induced by a viral infection. Even if several data might suggest that Kawasaki disease is an infection-related clinical syndrome, which can develop only in children with a predisposing genetic background, our knowledge on both the infectious agents involved and the genetic characteristics of children prone to the disease remains poor.


Download data is not yet available.
Abstract 663 | PDF Downloads 429 HTML Downloads 111


1. Kawasaki T. Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children. Arerugi 1967; 16:178-222. PMid: 6062087
2. Kawasaki T, Kosaki F, Okawa S, et al. A new infantile acute febrile mucocutaneous lymph node syndrome (MLNS) prevailing in Japan. Pediatrics 1974;54:271-6. PMid: 4153258
3. Newburger JW, Takahashi M, Gerber MA, et al. 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. Pediatrics 2004;114:1708-33. doi: 10.1542/peds.2004-2182 PMid: 15574639
4. Falcini F, Capannini S, Rigante D. Kawasaki syndrome: an intriguing disease with numerous unsolved dilemmas. Pediatr Rheumatol Online J 2011;9:17. doi: 10.1186/1546-0096-9-17 PMid: 21774801
5. Amano S, Hazama F, Kubagawa H, et al. General pathology of Kawasaki disease on the morphological alterations corresponding to the clinical manifestations. Acta Pathol Jpn 1980;30:681-94. PMid: 7446109
6. Hamashima Y. Kawasaki disease. Heart Vessels Suppl 1985;1:271-6. doi: 10.1007/BF02072407 PMid: 3843587
7. Suzuki A, Miyagawa-Tomita S, Komatsu K, et al. Active remodeling of the coronary arterial lesions in the late phase of Kawasaki disease: immunohistochemical study. Circulation 2000;101:2935-41. doi: 10.1161/01.cir.101.25.2935 PMid: 10869266
8. De Rosa G, Pardeo M, Rigante D. Current recommendations for the pharmacologic therapy in Kawasaki syndrome and management of its cardiovascular complications. Eur Rev Med Pharmacol Sci 2007;11:301-8. PMid: 18074939
9. Marchesi A, Tarissi De Jacobis I, Rigante D, et al. Kawasaki disease: guidelines of the Italian Society of Pediatrics, part I - definition, epidemiology, etiopathogenesis, clinical expression and management of the acute phase. Ital J Pediatr 2018;44(1):102. doi: 10.1186/s13052-018-0536-3 PMid: 30157897
10. Marchesi A, Tarissi De Jacobis I, Rigante D, et al. 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. Ital J Pediatr 2018;44(1):103. doi: 10.1186/s13052-018-0529-2 PMid: 30157893
11. Rigante D, Valentini P, Rizzo D, et al. Responsiveness to intravenous immunoglobulins and occurrence of coronary artery abnormalities in a single-center cohort of Italian patients with Kawasaki syndrome. Rheumatol Int 2010;30:841-6. doi: 10.1007/s00296-009-1337-1 PMid: 20049445
12. Rigante D, Andreozzi L, Fastiggi M, et al. Critical overview of the risk scoring systems to predict non-responsiveness to intravenous immunoglobulin in Kawasaki syndrome. Int J Mol Sci 2016;17:278. doi: 10.3390/ijms17030278 PMid: 26927060
13. Falcini F, Ozen S, Magni-Manzoni S, et al. Discrimination between incomplete and atypical Kawasaki syndrome versus other febrile diseases in childhood: results from an international registry-based study. Clin Exp Rheumatol 2012;30:799-804. PMid: 23020938
14. Stabile A, Bertoni B, Ansuini V, et al. The clinical spectrum and treatment options of macrophage activation syndrome in the pediatric age. Eur Rev Med Pharmacol Sci 2006;10:53-9. PMid: 16705949
15. Principi N, Rigante D, Esposito S. The role of infection in Kawasaki syndrome. J Infect 2013;67:1-10. doi: 10.1016/j.jinf.2013.04.004. PMid: 23603251
16. Rigante D, Tarantino G, Valentini P. Non-infectious makers of Kawasaki syndrome: tangible or elusive triggers? Immunol Res 2016;64:51-4. doi: 10.1007/s12026-015-8679-4 PMid: 26232895
17. Rowley AH, Shulman ST. The epidemiology and pathogenesis of Kawasaki disease. Front Pediatr. 2018;6:374. doi: 10.3389/fped.2018.00374 PMid: 30619784
18. Burns JC, Herzog L, Fabri O, et al. Seasonality of Kawasaki disease: a global perspective. PLoS One 2013;8(9):e74529. doi: 10.1371/journal.pone.0074529 PMid: 24058585
19. Shimizu D, Hoshina T, Kawamura M, et al. Seasonality in clinical courses of Kawasaki disease. Arch Dis Child 2019;104:694-6. doi: 10.1136/archdischild-2018-315267 PMid: 30413486
20. Turnier JL, Anderson MS, Heizer HR, et al. Concurrent respiratory viruses and Kawasaki disease. Pediatrics. 2015;136:e609-14. doi: 10.1542/peds.2015-0950 PMid: 26304824
21. Jordan-Villegas A, Chang ML, Ramilo O, et al. Concomitant respiratory viral infections in children with Kawasaki disease. Pediatr Infect Dis J 2010;29:770-2. doi: 10.1097/INF.0b013e3181dba70b PMid: 20354462
22. Benseler SM, McCrindle BW, Silverman ED, et al. Infections and Kawasaki disease: implications for coronary artery outcome. Pediatrics 2005;116:e760-6. doi: 10.1542/peds.2005-0559 PMid: 16322132
23. Matsuno S, Utagawa E, Sugiura A. Association of rotavirus infection with Kawasaki syndrome. J Infect Dis 1983;148:177. doi: 10.1093/infdis/148.1.177 PMid: 6309994
24. Johnson D, Azimi P. Kawasaki disease associated with Klebsiella pneumoniae bacteremia and parainfluenza type 3 virus infection. Pediatr Infect Dis J 1985;4:100-3. doi: 10.1097/00006454-198501000-00024 PMID: 2982131
25. Embil JA, McFarlane ES, Murphy DM, et al. Adenovirus type 2 isolated from a patient with fatal Kawasaki disease. Can Med Assoc J 1985;132:1400. PMid: 4005729
26. Jaggi P, Kajon AE, Mejias A, et al. Human adenovirus infection in Kawasaki disease: a confounding bystander? Clin Infect Dis 2013;56:58-64. doi: 10.1093/cid/cis807 PMid: 23011145
27. Song E, Kajon AE, Wang H, et al. Clinical and virologic characteristics may aid distinction of acute adenovirus disease from Kawasaki disease with incidental adenovirus detection. J Pediatr 2016;170:325-30. doi: 10.1016/j.jpeds.2015.11.021 PMid: 26707621
28. Okano M, Luka J, Thiele GM, et al. Human herpesvirus 6 infection and Kawasaki disease. J Clin Microbiol 1989;27:2379-80. PMid: 2555393
29. Hagiwara K, Komura H, Kishi F, Kaji T, Yoshida T. Isolation of human herpesvirus-6 from an infant with Kawasaki disease. Eur J Pediatr 1992;151:867-8. PMid: 1334835
30. Holm JM, Hansen LK, Oxhøj H. Kawasaki disease associated with parvovirus B19 infection. Eur J Pediatr 1995;154:633-4. doi: 10.1007/BF02079066 PMid: 7588963
31. Usta Guc B, Cengiz N, Yildirim SV, et al. Cytomegalovirus infection in a patient with atypical Kawasaki disease. Rheumatol Int 2008;28:387-9. doi: 10.1007/s00296-007-0440-4 PMid: 17717671
32. Kanegane H, Tsuji T, Seki H, et al. Kawasaki disease with a concomitant primary Epstein-Barr virus infection. Acta Paediatr Jpn 1994;36 713-6. doi: 10.1111/j.1442-200x.1994.tb03277.x PMid: 7871990
33. Kikuta H, Matsumoto S, Yanase Y, et al. Recurrence of Kawasaki disease and Epstein-Barr virus infection. J Infect Dis 1990;162:1215. doi 10.1093/infdis/162.5.1215 PMid: 2172398
34. Kuijpers TW, Herweijer TJ, Scholvinck L, et al. Kawasaki disease associated with measles virus infection in a monozygotic twin. Pediatr Infect Dis J 2000;19:350-3. doi: 10.1097/00006454-200004000-00018 PMid: 10783028
35. Shingadia D, Bose A, Booy R. Could a herpesvirus be the cause of Kawasaki disease? Lancet Infect Dis 2002;2:310-3. doi: 10.1016/s1473-3099(02)00265-7 PMid: 12062997
36. Lee DH, Huang HP. Kawasaki disease associated with chickenpox: report of two sibling cases. Acta Paediatr Taiwan 2004;45:94-6. PMid: 15335119
37. Joshi AV, Jones KD, Buckley AM, et al. Kawasaki disease coincident with influenza A H1N1/09 infection. Pediatr Int 2011;53:e1-2. doi: 10.1111/j.1442-200X.2010.03280.x PMid: 21342333
38. Huang X, Huang P, Zhang L, et al. Influenza infection and Kawasaki disease. Rev Soc Bras Med Trop 2015;48:243-8. doi: 10.1590/0037-8682-0091-2015 PMid: 26108000
39. Rigante D, Cantarini L, Piastra M, et al. Kawasaki syndrome and concurrent Coxsackie-virus B3 infection. Rheumatol Int 2012;32:4037-40. doi: 10.1007/s00296-010-1613-0 PMid: 21052673
40. Catalano-Pons C, Giraud C, Rozenberg F, et al. Detection of human bocavirus in children with Kawasaki disease. Clin Microbiol Infect 2007;13:1220-2. doi: 10.1111/j.1469-0691.2007.01827.x PMid: 17850342
41. Santos RA, Nogueira CS, Granja S, et al. Kawasaki disease and human bocavirus-potential association? J Microbiol Immunol Infect 2011;44:235-7. doi: 10.1016/j.jmii.2011.01.016 PMid: 21524620
42. Bajolle F, Meritet JF, Rozenberg F, et al. Markers of a recent bocavirus infection in children with Kawasaki disease: "a year prospective study". Pathol Biol (Paris) 2014;62:365-8. doi: 10.1016/j.patbio.2014.06.002 PMid: 25193448
43. Thissen JB, Isshiki M, Jaing C, et al. A novel variant of torque teno virus 7 identified in patients with Kawasaki disease. PLoS One 2018;13(12):e0209683. doi: 10.1371/journal.pone.0209683 PMid: 30592753
44. Maggio MC, Fabiano C, Corsello G. Kawasaki disease triggered by EBV virus in a child with familial Mediterranean fever. Ital J Pediatr 2019;45:129. doi: 10.1186/s13052-019-0717-8 PMid: 31627741
45. Maggio MC, Cimaz R, Alaimo A, et al. Kawasaki disease triggered by parvovirus infection: an atypical case report of two siblings. J Med Case Rep 2019;13:104. doi: 10.1186/s13256-019-2028-5 PMid: 31014402
46. Wang J, Sun F, Deng HL, et al. Influenza A (H1N1) pdm09 virus infection in a patient with incomplete Kawasaki disease: A case report. Medicine (Baltimore) 2019;98(15):e15009. doi: 10.1097/MD.0000000000015009 PMid: 30985646
47. Chang LY, Lu CY, Shao PL, et al. Viral infections associated with Kawasaki disease. J Formos Med Assoc 2014;113:148-54. doi: 10.1016/j.jfma.2013.12.008 PMid: 24495555
48. Esper F, Shapiro ED, Weibel C, et al. Association between a novel human coronavirus and Kawasaki disease. J Infect Dis 2005;191:499-502. doi: 10.1086/428291 PMid: 15655771
49. Dominguez SR, Anderson MS, Glodé MP, et al. Blinded case-control study of the relationship between human coronavirus NL63 and Kawasaki syndrome. J Infect Dis 2006;194:1697-01. doi: 10.1086/509509 PMid: 17109341
50. Shirato K, Imada Y, Kawase M, et al. Possible involvement of infection with human coronavirus 229E, but not NL63, in Kawasaki disease. J Med Virol 2014;86:2146-53. doi: 10.1002/jmv.23950 PMid: 24760654
51. Jones VG, Mills M, Suarez D, et al. COVID-19 and Kawasaki disease: novel virus and novel case. Hosp Pediatr 2020;10:537-40. doi: 10.1542/hpeds.2020-0123 PMid: 32265235
52. Rivera-Figueroa EI, Santos R, Simpson S, et al. Incomplete Kawasaki Disease in a child with Covid-19. Indian Pediatr 2020 May 9:S097475591600179. PMid: 32393680
53. Verdoni L, Mazza A, Gervasoni A, et al. An outbreak of severe Kawasaki-like disease at the Italian epicentre of the SARS-CoV-2 epidemic: an observational cohort study. Lancet 2020;395(10239): 1771-8. doi: 10.1016/S0140-6736(20)31103-X PMid: 32410760
54. Dufort EM, Koumans EH, Chow EJ, et al. Multisystem inflammatory syndrome in children in New York State. N Engl J Med 2020 Jun 29. doi: 10.1056/NEJMoa2021756 PMid: 32598830
55. Rowley AH, Baker SC, Shulman ST, et al. RNA-containing cytoplasmic inclusion bodies in ciliated bronchial epithelium months to years after acute Kawasaki disease. PLoS One 2008;3:e1582. doi: 10.1371/journal.pone.0001582 PMid: 18270572
56. Rowley AH, Baker SC, Shulman ST, et al. Ultrastructural, immunofluorescence, and RNA evidence support the hypothesis of a "new" virus associated with Kawasaki disease. J Infect Dis 2011;203:1021-30. doi: 10.1093/infdis/jiq136 PMid: 21402552
57. L'Huillier AG, Brito F, Wagner N, et al. Identification of viral signatures using high-throughput sequencing on blood of patients with Kawasaki disease. Front Pediatr 2019;7:524. doi: 10.3389/fped.2019.00524 PMid: 31921732
58. Esposito S, Polinori I, Rigante D. The gut microbiota-host partnership as a potential driver of Kawasaki syndrome. Front Pediatr 2019;7:124. doi: 10.3389/fped.2019.00124 PMid: 31024869
59. Marrani E, Burns JC, Cimaz R. How should we classify Kawasaki disease? Front Immunol 2018;9:2974. doi: 10.3389/fimmu.2018.02974 PMid: 30619331
60. Rigante D. A systematic approach to autoinflammatory syndromes: a spelling booklet for the beginner. Expert Rev Clin Immunol 2017;13:571-97. doi: 10.1080/1744666X.2017.1280396 PMid: 28064547
61. Rigante D. The broad-ranging panorama of systemic autoinflammatory disorders with specific focus on acute painful symptoms and hematologic manifestations in children. Mediterr J Hematol Infect Dis 2018;10(1):e2018067. doi: 10.4084/MJHID.2018.067 PMid: 30416699
62. Rigante D, Frediani B, Galeazzi M, et al. From the Mediterranean to the sea of Japan: the transcontinental odyssey of autoinflammatory diseases. Biomed Res Int 2013;2013:485103. doi: 10.1155/2013/485103 PMid: 23971037

Most read articles by the same author(s)