Abstract 20: Kawasaki Disease In France: Incomplete Forms Are Frequent And Associated With A High Frequency Of Cardiac Complications

KD is the main vasculitis affecting children Methods: Institutional physicians received information on a national registry for KD. All patients suspected with KD and seen since 2011 were eligible to enter the study. An eCRF was implemented in a web database.The included patients without the AHA international criteria were reviewed by an experts9 committee. Results: 468 cases were entered by 84 physicians from 65 centers. The AHA classification gave: 280 complete KD, and 73 incomplete KD. An expert consensus classified 48 other patients as probable leading to 401 patients considered as KD (M229/F72). 67 were excluded (incomplete data or doubful). The median age at diagnosis was 3.1y (2m-14y). Their ethnical backgrounds were: European Caucasian 67%, Eastern Caucasian/North African 15%, afro-Caribbean 13%, Asian 4% and mixed ancestry 1%. The clinical symptoms were (%): conjunctivitis 84, cheilitis 82, diffuse exanthema 74, modification of the extremities 73, oral erythema 66, cervical adenopathy 52, raspberry tongue 49, seat erythema 26, perineal desquamation 18 and BCG erythema 5. The cardiac complications were: coronary dilatation 30%, pericarditis 15%, coronary aneurysm 4%, and myocarditis 3% (1 death). 392/401 (98%) patients received IVIG, 64 (21%) and 5 required 2 and 3 courses. The mean treatment delay was 6 days. The factors associated with the coronary abnormalities were: male gender (p=0.01), young KD onset age (p=0.03), and resistance to IVIG (p=0.03). conclusion: KD diagnosis remains challenging and overdiagnosis represents at least 10% of cases in this registry. Incomplete forms of KD account for 37 % and are associated with coronary dilatation/aneurysm (34%; p