145 - RESPONSE OF MEDIUM AND GIANT CORONARY ARTERY ANEURYSMS TO TREATMENT INTENSIFICATION WITH INFLIXIMAB WITH RESPECT TO IT'S TIME OF ADMINISTRATION AFTER INTRAVENOUS IMMUNOGLOBULIN: RETROSPECTIVE ANALYSIS

BACKGOUND In Kawasaki Disease (KD) intravenous immunoglobulin (IVIG) reduces the incidence of coronary artery aneurysms (CAA) to 5%. Treatment intensification with corticosteroids or infliximab (IFX) has been proposed for children presenting with CAA at diagnosis. There is paucity of data on the effect of IFX, after IVIG, on medium to large CAAs with respect to the time of administration of IFX and CAA regression.  

AIMS AND

Objectives: To determine the duration to diminution in size of medium and large CAAs with respect to time of administration of  IFX after IVIG.

 RATIONALE: Endothelial inflammation continues for a longer time than clinically evident. There is limited data on timing of IFX administration as upfront or rescue therapy.

MATERIALS AND METHODS This is a retrospective analysis of data of children with KD who received  IFX (after IVIG) with medium to large CAAs,  admitted between January 2016 to September 2022 at Institute of Child Health, Kolkata.  Medium to large CAAs were present at diagnosis or developed on follow up echoes post IVIG. CAAs were classified as per AHA 2017 guidelines. The   timing of IFX administration was classified as ≤3 days, 4 to 7 days, ≥8 days after the first dose of IVIG. Data noted were size of CAAs on serial follow up echoes, and the duration to response of CAA was noted in three categories: reduction of CAA by atleast 1z score, reduction by 50% and complete regression as defined by CAA z score ≤2z. 

RESULTS

From January 2016 to November 2022 a total 219 children were diagnosed as Kawasaki disease. 28 (10.9%) patients had CAA at diagnosis or developed CAA after IVIG. 20 of these received IFX after IVIG. Other 8 were excluded as they received combination therapy of IFX with Steroids +/-  Cyclosporine.  

13 had medium CAA. All of them had shown complete regression of CAA with a median duration of 180 days.

7 had large CAA of which  3 had complete regression of CAA over 2 years while 4 others had persistent CAA.

Duration to response with respect to time of administration of IFX after IVIG in Medium CAA (Table 1) and in Large CAA (Table 2) is described below.

Conclusion:
From our cohort we noted therapy intensification with Infliximab  results in regression of CAAs. Medium coronary artery aneurysms completely regressed even when Infliximab  is administered late. Even few giant CAAs completely regressed with Infliximab following IVIG