Skip to main content

Advertisement

Log in

The factors affecting the disease course in Kawasaki disease

  • Cohort Studies
  • Published:
Rheumatology International Aims and scope Submit manuscript

Abstract

The aim of this study was to review the characteristics of patients with Kawasaki disease (KD) from Turkey and to assess the performance of the Kobayashi score (KS), Harada score (HS), Formosa score (FS), Egami score (ES) and other parameters in predicting intravenous immunoglobulin (IVIG) resistance and coronary artery involvement (CAI) in the Turkish population. Patients who were diagnosed as being in the acute phase of KD at Hacettepe University Faculty of Medicine (Ankara, Turkey) between June 2007 and January 2016 reviewed retrospectively, and those between January 2016 and February 2018 reviewed prospectively, were included in this cohort study. A total of 100 patients with KD were included in this study. Statistical Package for Social Sciences for Windows 22.0 (SPSS Inc, Chicago, IL, USA) was used for statistical analysis. Eighty-five patients (85%) responded to IVIG treatment, whereas 15 (5 female, 10 male) were IVIG resistant. CAI was detected in echocardiography at diagnosis in 31 (31%) (9 female; 22 male) patients. For predicting IVIG resistance, KS, ES, FS, and HS had sensitivity of 82.1%, 26.7%, 30.8%, 69.2% and specificity of 35.7%, 94%, 51.2%, 45.8%, respectively. For the association with CAI occurrence, the sensitivities were 17.2%, 3.3%, 35.7%, 70.4% and the specificities were 78.5%, 88.4%, 49.3%, 49.3% for the aforementioned scores, respectively. The multivariate analysis showed white blood cell (WBC) count [Odd’s ratio (OR) 4.1; 95% confidence interval (CI) 1.26–13.23; p = 0.019] and hematocrit (OR 3.8; 95% CI 1.15–12.4; p = 0.028), as independent predictors of CAI while gamma-glutamyl transferase (GGT) level (OR 5.7; 95% CI 1.73–27.51; p = 0.018) was detected as the only independent predictor of IVIG resistance. This is the first study from Turkey in KD to evaluate the association of the scoring systems for IVIG resistance and CAI. The risk scoring systems in KD did not predict the risk for IVIG resistance and were not associated with CAI in Turkish population.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1

Similar content being viewed by others

Abbreviations

ALT:

Alanine aminotransferase

AST:

Aspartate aminotransferase

CAI:

Coronary artery involvement

CRP:

C-reactive protein

ES:

Egami score

ESR:

Erythrocyte sedimentation rate

FS:

Formosa score

GGT:

Gamma-glutamyl transferase

HS:

Harada score

IL:

Interleukin

IVIG:

Intravenous immunoglobulin

IQR:

Interquartile range

KD:

Kawasaki disease

KS:

Kobayashi score

MP:

Methylprednisolone

OR:

Odd’s ratio

ROC:

Receiver operating characteristic

SPSS:

Statistical Package for Social Sciences

WBC:

White blood cell

References

  1. McCrindle BW, Rowley AH, Newburger JW, Burns JC, Bolger AF, Gewitz M et al (2017) Diagnosis, treatment, and long-term management of Kawasaki disease: a scientific statement for health professionals from the American Heart Association. Circulation 135(17):e927–e999. https://doi.org/10.1161/cir.0000000000000484

    Article  Google Scholar 

  2. Batu ED, Ozen S (2012) Pediatric vasculitis. Curr Rheumatol Rep 14(2):121–129. https://doi.org/10.1007/s11926-011-0232-4

    Article  CAS  PubMed  Google Scholar 

  3. Eleftheriou D, Batu ED, Ozen S, Brogan PA (2015) Vasculitis in children. Nephrol Dial Transpl 30(Suppl 1):i94–i103. https://doi.org/10.1093/ndt/gfu393

    Article  CAS  Google Scholar 

  4. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F et al (2013) 2012 revised international Chapel Hill consensus conference nomenclature of vasculitides. Arthritis Rheum 65(1):1–11. https://doi.org/10.1002/art.37715

    Article  CAS  PubMed  Google Scholar 

  5. Batu ED, Ozen S (2015) Vasculitis: do we know more to classify better? Pediatr Nephrol 30(9):1425–1432. https://doi.org/10.1007/s00467-014-3015-0

    Article  PubMed  Google Scholar 

  6. Burns JC, Franco A (2015) The immunomodulatory effects of intravenous immunoglobulin therapy in Kawasaki disease. Expert Rev Clin Immunol 11(7):819–825. https://doi.org/10.1586/1744666x.2015.1044980

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  7. Aoyagi R, Hamada H, Sato Y, Suzuki H, Onouchi Y, Ebata R et al (2015) Study protocol for a phase III multicentre, randomised, open-label, blinded-end point trial to evaluate the efficacy and safety of immunoglobulin plus cyclosporin A in patients with severe Kawasaki disease (KAICA Trial). BMJ Open 5(12):e009562. https://doi.org/10.1136/bmjopen-2015-009562

    Article  PubMed  PubMed Central  Google Scholar 

  8. Marrani E, Burns JC, Cimaz R (2018) How should we classify Kawasaki disease? Front Immunol 9:2974. https://doi.org/10.3389/fimmu.2018.02974

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  9. Egami K, Muta H, Ishii M, Suda K, Sugahara Y, Iemura M et al (2006) Prediction of resistance to intravenous immunoglobulin treatment in patients with Kawasaki disease. J Pediatr 149(2):237–240. https://doi.org/10.1016/j.jpeds.2006.03.050

    Article  CAS  PubMed  Google Scholar 

  10. Kobayashi T, Inoue Y, Morikawa A (2008) Risk stratification and prediction of resistance to intravenous immunoglobulin in Kawasaki disease. Nihon Rinsho 66(2):332–337

    PubMed  Google Scholar 

  11. Harada K (1991) Intravenous gamma-globulin treatment in Kawasaki disease. Acta Paediatr Jpn 33(6):805–810

    Article  CAS  PubMed  Google Scholar 

  12. Lin MT, Chang CH, Sun LC, Liu HM, Chang HW, Chen CA et al (2016) Risk factors and derived formosa score for intravenous immunoglobulin unresponsiveness in Taiwanese children with Kawasaki disease. J Formos Med Assoc 115(5):350–355. https://doi.org/10.1016/j.jfma.2015.03.012

    Article  CAS  PubMed  Google Scholar 

  13. Sleeper LA, Minich LL, McCrindle BM, Li JS, Mason W, Colan SD et al (2011) Evaluation of Kawasaki disease risk-scoring systems for intravenous immunoglobulin resistance. J Pediatr 158(5):831–835. https://doi.org/10.1016/j.jpeds.2010.10.031

    Article  PubMed  Google Scholar 

  14. Fabi M, Andreozzi L, Corinaldesi E, Bodnar T, Lami F, Cicero C et al (2019) Inability of Asian risk scoring systems to predict intravenous immunoglobulin resistance and coronary lesions in Kawasaki disease in an Italian cohort. Eur J Pediatr 178(3):315–322. https://doi.org/10.1007/s00431-018-3297-5

    Article  CAS  PubMed  Google Scholar 

  15. Loomba RS, Raskin A, Gudausky TM, Kirkpatrick E (2016) Role of the Egami Score in predicting intravenous immunoglobulin resistance in Kawasaki disease among different ethnicities. Am J Ther 23(6):e1293–e1299. https://doi.org/10.1097/mjt.0000000000000045

    Article  PubMed  Google Scholar 

  16. Song R, Yao W, Li X (2017) Efficacy of four scoring systems in predicting intravenous immunoglobulin resistance in children with Kawasaki Disease in a Children’s Hospital in Beijing, North China. J Pediatr 184:120–124. https://doi.org/10.1016/j.jpeds.2016.12.018

    Article  PubMed  Google Scholar 

  17. Shin J, Lee H, Eun L (2017) Verification of current risk scores for Kawasaki disease in Korean children. J Korean Med Sci 32(12):1991–1996. https://doi.org/10.3346/jkms.2017.32.12.1991

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  18. de Graeff N, Groot N, Ozen S, Eleftheriou D, Avcin T, Bader-Meunier B et al (2019) European consensus-based recommendations for the diagnosis and treatment of Kawasaki disease—the SHARE initiative. Rheumatology 58(4):672–682. https://doi.org/10.1093/rheumatology/key344

    Article  PubMed  Google Scholar 

  19. Ozen S, Sonmez HE, Demir S (2018) Pediatric forms of vasculitis. Best Pract Res Clin Rheumatol 32(1):137–147. https://doi.org/10.1016/j.berh.2018.09.007

    Article  PubMed  Google Scholar 

  20. Ozen S, Batu ED (2018) Vasculitis pathogenesis: can we talk about precision medicine? Front Immunol 9:1892. https://doi.org/10.3389/fimmu.2018.01892

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  21. Arane K, Mendelsohn K, Mimouni M, Mimouni F, Koren Y, Brik Simon D et al (2018) Japanese scoring systems to predict resistance to intravenous immunoglobulin in Kawasaki disease were unreliable for Caucasian Israeli children. Acta Paediatr 107(12):2179–2184. https://doi.org/10.1111/apa.14418

    Article  CAS  PubMed  Google Scholar 

  22. Wang Y, Li Z, Hu G, Hao S, Deng X, Huang M et al (2016) Unique molecular patterns uncovered in Kawasaki disease patients with elevated serum gamma glutamyl transferase levels: implications for intravenous immunoglobulin responsiveness. PLoS One 11(12):e0167434. https://doi.org/10.1371/journal.pone.0167434

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  23. Belay ED, Maddox RA, Holman RC, Curns AT, Ballah K, Schonberger LB (2006) Kawasaki syndrome and risk factors for coronary artery abnormalities: United States, 1994–2003. Pediatr Infect Dis J 25(3):245–249. https://doi.org/10.1097/01.inf.0000202068.30956.16

    Article  PubMed  Google Scholar 

  24. Sudo D, Monobe Y, Yashiro M, Sadakane A, Uehara R, Nakamura Y (2010) Case-control study of giant coronary aneurysms due to Kawasaki disease: the 19th nationwide survey. Pediatr Int 52(5):790–794. https://doi.org/10.1111/j.1442-200x.2010.03161.x

    Article  PubMed  Google Scholar 

  25. Rigante D, Andreozzi L, Fastiggi M, Bracci B, Natale MF, Esposito S (2016) Critical overview of the risk scoring systems to predict non-responsiveness to intravenous immunoglobulin in Kawasaki syndrome. Int J Mol Sci 17(3):278. https://doi.org/10.3390/ijms17030278

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  26. Clark DE, Denby KJ, Kaufman LM, Fill MA, Piya B, Krishnaswami S et al (2018) Predictors of intravenous immunoglobulin nonresponse and racial disparities in Kawasaki disease. Pediatr Infect Dis J37(12):1227–1234. https://doi.org/10.1097/inf.0000000000002019

    Article  Google Scholar 

  27. Chantasiriwan N, Silvilairat S, Makonkawkeyoon K, Pongprot Y, Sittiwangkul R (2018) Predictors of intravenous immunoglobulin resistance and coronary artery aneurysm in patients with Kawasaki disease. Paediatr Int Child Health 38(3):209–212. https://doi.org/10.1080/20469047.2018.1471381

    Article  PubMed  Google Scholar 

  28. Jakob A, Whelan J, Kordecki M, Berner R, Stiller B, Arnold R et al (2016) Kawasaki disease in Germany: a prospective, population-based study adjusted for underreporting. Pediatr Infect Dis J 35(2):129–134. https://doi.org/10.1097/inf.0000000000000953

    Article  PubMed  Google Scholar 

  29. Kuo HC, Yang KD, Chang WC, Ger LP, Hsieh KS (2012) Kawasaki disease: an update on diagnosis and treatment. Pediatr Neonatol. 53(1):4–11. https://doi.org/10.1016/j.pedneo.2011.11.003

    Article  PubMed  Google Scholar 

  30. Sag E, Batu ED, Ozen S (2017) Childhood systemic vasculitis. Best Pract Res Clin Rheumatol 31(4):558–575. https://doi.org/10.1016/j.berh.2017.11.009

    Article  PubMed  Google Scholar 

  31. Huang SK, Lin MT, Chen HC, Huang SC, Wu MH (2013) Epidemiology of Kawasaki disease: prevalence from national database and future trends projection by system dynamics modeling. J Pediatr 163(1):126–131. https://doi.org/10.1016/j.jpeds.2012.12.011

    Article  PubMed  Google Scholar 

  32. Lin MT, Wu MH (2017) The global epidemiology of Kawasaki disease: review and future perspectives. Glob Cardiol Sci Pract 2017(3):201720

    Google Scholar 

Download references

Funding

There is no funding source.

Author information

Authors and Affiliations

Authors

Contributions

Manuscript EAA; literature search EAA, EDB, SO; figure EAA, SO; study desing EAA, SO; data collection EAA, EDB, HES, SD, ZSA, ES, İE, DA, YB, SO; data analysis EAA, SO; data interpretation EAA, YB, SO; writing EAA, EDB, SO. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Seza Ozen.

Ethics declarations

Conflict of interest

The authors declare no conflict of interest.

Research involving human participants and/or animals

When the patients admitted to the hospital, the parents gave a general consent approving anonymous data use for academic purpose.

Informed consent

The written consents from the patient families were obtained according to the Declaration of Helsinki (1964) and the study was approved by the ethics committee of Hacettepe University (GO-16/45-15; approval date, 1st March 2016).

Ethics approval and consent to participate

The written consents from the patient families were obtained according to the Declaration of Helsinki (1964) and the study was approved by the ethics committee of Hacettepe University (GO-16/45-15; approval date, 1st March 2016).

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Electronic supplementary material

Below is the link to the electronic supplementary material.

Supplementary material 1 (DOCX 18 kb)

Supplementary material 2 (DOCX 17 kb)

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Arslanoglu Aydin, E., Ertugrul, I., Bilginer, Y. et al. The factors affecting the disease course in Kawasaki disease. Rheumatol Int 39, 1343–1349 (2019). https://doi.org/10.1007/s00296-019-04336-2

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00296-019-04336-2

Keywords

Navigation