Outcomes of Kawasaki Disease in Families


  •   Toshimasa Nakada


An epidemiological study showed that a positive family history of Kawasaki disease (KD) was a risk factor for intravenous immunoglobulin (IVIG) therapy resistance, coronary artery lesions (CALs), and KD recurrence. However, real-world outcomes of KD patients with a family history remain unclear. The objective of this study was to elucidate the outcomes of KD patients with a family history in the era of 2 g/kg IVIG therapy. This retrospective study included data from 201 KD patients who underwent acute-phase treatment from January 2009 to June 2020, with 184 (91.5%) receiving 2 g/kg IVIG therapy. The patients were divided into 13 (family group) with and 188 (nonfamily group) without a family history of KD. The rates of IVIG resistance (8.3% vs. 22.1%, P = 0.315), rescue therapy (8.3% vs. 12.8%, P = 1.000), CALs (0.0% vs. 2.7%, P = 1.000), and KD recurrence (0.0% vs. 3.2%, P = 1.000) were similar between the family and nonfamily groups.

Keywords: Family, Intravenous immunoglobulin therapy, Kawasaki disease, Outcomes.



Onouchi Y, Gunji T, Burns JC, Shimizu C, Newburger JW, Yashiro M, et al. ITPKC functional polymorphism associated with Kawasaki disease susceptibility and formation of coronary artery aneurysms. Nat Genet. 2008 January; 40 (1): 35–42. doi:10.1038/ng.2007.59.

Cook DH, Antia A, Attie F, Gersony WM, Kamiya T, Kato H, et al. Results from an international survey of Kawasaki disease in 1979–82. Can J Cardiol 1989; 5: 389–94. [PubMed: 2605549].

Fujita Y, Nakamura Y, Sakata K, Hara N, Kobayashi M, Nagai M, et al. Kawasaki disease in families. Pediatrics 1989; 84: 666–9. [PubMed: 2780128].

Uehara R, Yashiro M, Nakamura Y, Yanagawa H. Kawasaki disease in parents and children. Acta Paediatr 2003; 92: 694–7. [PubMed: 12856980] doi: 10.1080/08035320310002768.

Dergun M, Kao A, Hauger SB, Newburger JW, Burns JC. Familial occurrence of Kawasaki syndrome in North America. Arch Pediatr Adolesc Med 2005; 159: 876–81. doi:10.1001/archpedi.159.9.876. [PubMed: 16143748].

Uehara R, Yashiro M, Nakamura Y, Yanagawa H. Clinical features of patients with Kawasaki disease whose parents had the same disease. Arch Pediatr Adolesc Med 2004; 158 (12): 1166–9. doi: 10.1001/archpadi.158.12.1166.

Ayusawa M, Sonobe T, Uemura S, Ogawa S, Nakamura Y, Kiyosawa N, et al. Revision of diagnostic guidelines for Kawasaki disease (the 5th revised edition). Pediatr Int 2005; 47: 232–4. https://doi.org/10.1111/j.1442-200x.2005.02033.

Fukazawa R, Kobayashi J, Ayusawa M, Hamada H, Miura M, Mitani Y, et al. JCS/JSCS 2020 Guideline on Diagnosis and Management of Cardiovascular Sequelae in Kawasaki Disease. Circ J 2020; 84: 1348–1407. doi:10.1253/circj.CJ-19-1094.

Rearch Committee of the Japanese Society of Pediatric Cardiology; Cardiac Surgery Committee for Development of Guidelines for Medical Treatment of Acute Kawasaki Disease. Guidelines for medical treatment of acute Kawasaki disease: report of the Research Committee of the Japanese Society of Pediatric Cardiology and Cardiac Surgery (2012 revised version). Pediatr Int 2014; 56: 135–58. https://doi.org/10.1111/ped.12317.

Hirata S, Nakamura Y, Yanagawa H. Incidence rate of recurrent Kawasaki disease and related risk factors: from the results of nationwide surveys of Kawasaki disease in Japan. Acta Paediatr. 2001; 90(1): 40–4. https://doi.org/10.1111/j.1651-2227.2001.tb00253.x.

Nakada T. Usefulness of an initial single intravenous immunoglobulin infusion with delayed use of aspirin against Kawasaki disease relapse: a single-center retrospective study. Journal of Advanced Research in Medicine 2019; 6 (1): 18–24. https://doi.org/10.24321/2349.7181.201905.

Nakada T. Acute Phase Treatment and Medium-Term Outcomes in Kawasaki Disease. European Journal of Medical and Health Sciences 2020; 2 (4), August: 1–7. doi: http://dx.doi.org/10.24018/ejmed.2020.2.4.419.

Nakada T. Effects of anti-inflammatory drugs on intravenous immunoglobulin therapy in the acute phase of Kawasaki disease. Pediatr Cardiol 2015; 36: 335–9. DOI 10.1007/s00246-014-1010-7.

Kobayashi T, Inoue Y, Takeuchi K, OkadaY, Tamura K, Tomomasa T, et al. Prediction of intravenous immunoglobulin unresponsiveness in patients with Kawasaki disease. Circulation 2006; 113: 2606–12. http://www.circulationaha.org DOI:10.1161/CIRCULATIONAHA.105.592865.

Marume K, Noguchi T, Tateishi E, Morita Y, Miura H, Nishimura K, et al. Prognosis and Clinical Characteristics of Dilated Cardiomyopathy With Family History via Pedigree Analysis. Circ J 2020; 84: 1284–93. doi:10.1253/circj.CJ-19-1176.

McCrindle BW, Tierney ESS. Acute treatment for Kawasaki disease: challenges for current and future therapies. J Pediatr 2017; 184: 7–10. DOI: 10.1016/j.jpeds.2017.01.072.

Kuo HC, Guo MM, Lo MH, Hsieh KS, Huang YH. Effectiveness of intravenous immunoglobulin alone and intravenous immunoglobulin combined with high-dose aspirin in the acute stage of Kawasaki disease: study protocol for a randomized controlled trial. BMC Pediatr. 2018; 18: 200. https://doi.org/10.1186/s12887-018-1180-1.

Lau AC, Duong TT, Ito S, Yeung RS. Intravenous immunoglobulin and salicylate differentially modulate pathogenic processes leading to vascular damage in a model of Kawasaki disease. Arthritis Rheum 2009; 60: 2131–41. https://doi.org/10.1002/art.24660.

Cho HJ, Bak SY, Kim SY, Yoo R, Baek HS, Yang S, et al. High neutrophil: lymphocyte ratio is associated with refractory Kawasaki disease. Pediatr Int 2017; 59: 669–74. https://doi.org/10.1111/ped.13240|.

Nakada T. Acute phase treatment for prevention of coronary artery stenosis caused in Kawasaki disease: a single center retrospective study. Journal of Advanced Research in Medicine 2018; 5(4): 1–7. https://doi.org/10.24321/2349.7181.201818.

Kottek A, Shimizu C, Burns JC. Kawasaki disease in monozygotic twins. Pediatr Infect Dis J 2011; 30(12):1114–6. doi:10.1097/INF.0b013e31822ac4ff.

Zhang X, Sun J, Zhai S, Yang S. Kawasaki disease in two sets of monozygotic twins: is the etiology genetic or environmental? Pak J Med Sci 2013; 29(1):227–30.

Fukuda S, Ito S, Fujiwara M, Abe J, Hanaoka N, Fujimoto T, Katsumori H. Simultaneous development of Kawasaki disease following acute human adenovirus infection in monozygotic twins: a case report. Pediatr Rheumatol Online J 2017; 15(1):39.

Namita U, Saddiq MH, Ahamed Z. Simultaneous development of Kawasaki disease in identical twins: a case report. J Fam Med Prim Care 2019; 8(4):1481–2.

Nakada T: Intravenous Immunoglobulin Therapy for Prevention of Coronary Artery Stenosis Caused in Kawasaki Disease. International Journal of Advances in Medical Sciences 2018; 3 (6): 1–13. www.kibanresearchpublications.com.

Makino N, Nakamura Y, Yashiro M, Kosami K, Matsubara Y, Ae R, et al. Nationwide epidemiologic survey of Kawasaki disease in Japan, 2015–2016. Pediatr Int 2019; 61: 397–403. https://doi.org/10.1111/ped.13809.

Banday AZ, Bhattacharya D, Pandiarajan V, Singh S. Kawasaki disease in siblings in close temporal proximity to each other—what are the implications? Clinical Rheumatology Pulished online: 10 August 2020 https://doi.org/10.1007/s10067-020-05328-5.

Lee DH, Huang HP. Kawasaki disease associated with chickenpox: report of two sibling cases. Acta Paediatr Taiwan 2004; 45 (2): 94–6. [PMID: 15335119].

Kuijpers TW, Herweijer TJ, Schölvinck L, Wertheim-Van Dillen PM, Van de Veer EM. Kawasaki disease associated with measles virus infection in a monozygotic twin. Pediatr Infect Dis J 2000;19(4):350–3.

Rodó X, Curcoll R, Robinson M, Ballester J, Burns JC, Cayan DR, et al. Tropospheric winds from northeastern China carry the etiologic agent of Kawasaki disease from its source to Japan. Proc Natl Acad Sci USA 2014; 111(22):7952–7.


Download data is not yet available.


How to Cite
Nakada, T. (2020). Outcomes of Kawasaki Disease in Families. European Journal of Medical and Health Sciences, 2(5). https://doi.org/10.24018/ejmed.2020.2.5.494