Winner of the 2020 PReS/PRINTO call for grants, the study aims to analyze demographic, clinical, biological and echocardiographic variable associated with IVIg resistance in European and North Indian cohorts of KD children, to assess difference in clinical and treatment outcomes between European and North Indian KD cohorts and to assess the performance of Kobayashi and Kawanet scores in European and North Indian KD cohorts.
BACKGROUND and RATIONALE
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, a single infusion of 2 g/kg intravenous immunoglobulins (IVIG) along with aspirin, 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, since they proved adequate specificity (87%) but poor sensitivity (33%) in European, American and other Asian populations. Recently, the French Kawanet group have proposed an 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.
KIWI is a retrospective-prospective, observational, descriptive and multicenter study of patients with KD according to the criteria established by the American Heart Association (AHA). The core setting is represented by 1 Asian [Chandigarh (India)] and 4 European pediatric rheumatology centers [Firenze, Brescia and Trieste (Italy) and Bristol (United Kingdom)].
Data deriving from KD patients diagnosed in the past 5 years will be collected retrospectively by the revision of medical records, new KD diagnosis will be collected prospectively during the study period.
In the 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.
Please feel free to contact the PRINTO coordinating centre to get the slides and recording of the study Kick-off meeting, held on December 1st, 2021.
PI: Dr Maria Vincenza Mastrolia