European and North Indian Cohort of KaWasaki dIsease

Condition(s) studied

Study summary

Kawasaki disease (KD) is currently the leading cause of acquired heart diseases in children in developed countries. Cardiac involvement is the main determinant of the long-term prognosis of these patients, as coronary aneurisms (CAAs) may lead to ischemic heart disease and even sudden death. The current standard of care for KD has consistently reduced CAAs frequency from 25-30% to about 5%. Unfortunately, 10-20% of KD patients results resistant to standard treatment leading to a major risk of cardiac complications. Thus, scoring systems have been constructed in order to identify patients likely to be resistant to IVIG and who may benefit from more aggressive initial therapy. Different scoring scales developed by Kobayashi, Egami et Sano had shown a good sensitivity (77-86%) and specificity (67-86%) in predicting IVIG unresponsiveness in Japanese populations. However, their predictive value was not confirmed by subsequent studies in different ethnic populations. Recently, the French Kawanet group have proposed a IVIG unresponsiveness score that provided good sensitivity and acceptable specificity in a non-Asian KD population even if it was not subsequent validated by an external study. In our study population, the achievement of specificity and sensitivity values for both scores consistent with those reported by the original studies (sensitivity 70% and specificity 80% for Kobayashi and sensitivity 77% and specificity 60% for Kawanet), will be considered a success.

Eligibility

Primary outcome measure(s)

• IVIG resistance rate — through study completion, an average of 1 year
  IVIG resistance rate in patients with KD and stratification according to different ethnic groups.
• percentage of IVIG-resistant patients — through study completion, an average of 1 year
  percentage of IVIG-resistant patients who met the Kobayashi and KAWANET criteria, and specificity and sensitivity of the criteria within the different ethnic groups.

Trial sites (27)

More Meyer Children's Hospital IRCCS trials in Spain