503. Lack of coronary aneurysms during Kawasaki Disease (KD) correlates with higher levels of autoantibodies to both full form and spliced variant of immune regulator EDIL-3 (Del-1)


 
 
 Kawasaki disease is a rare multisystem inflammatory condition that mainly affects children under the age of 5 years. Current theory of the pathogenesis is that an infectious stimulus triggers an autoimmune reaction in a genetically susceptible child. Recent studies demonstrated an association with autoantibody response to EDIL3 (also known as DEL-1) in children with KD. EDIL3 is an extracellular matrix protein which is expressed both in macrophages and vascular endothelium. EDIL3, has an anti-inflammatory role by preventing leucocyte migration to inflammatory sites. Del-1 has two expression variants and genetic variants of Del-1 have been associated with the risk of intracranial aneurysms. Due to the physiologic plausibility for a role during KD, we chose to assess if autoantibodies against EDIL3 are seen in a larger cohort of children with KD and to assess if responses correlated to aneurysm formation.
 
 
 
 We assessed plasma samples from a cohort of febrile children, including Kawasaki disease with ELISA assay using recombinant EDIL3 using ELISAs.
 
 
 
 Time course samples of KD (pre-IVIG, post-IVIG and convalescent) from two different patient cohorts showed that there is increased autoantibody levels against EDIL3 in the post-IVIG samples, which is consistent with the commonality of antibodies. Contrary to prior findings, in comparison to febrile controls, autoantibodies were not higher in children with Kd. Correlation with signs of cardiac inflammation showed lower autoantibody levels.
 
 
 
 Auto-antibodies to EDIL3 are common in febrile children and, like other autoantibodies, are seen in IVIG formulation. Lower levels of these may be a susceptibility marker for KD or may be sequestered during aneurysm formation.
 
 
 
 All Authors: No reported disclosures.
