73 - EVALUATING THE PUBLISHED PREVALENCE OF GIANT ANEURYSMS IN KAWASAKI DISEASE ACROSS THE ARAB WORLD: A KAWARABI LITERATURE REVIEW

Background: The development of coronary artery aneurysms (CAA) is the main severe complication of KD, with giant aneurysms (GiAn) posing a potentially fatal risk. While numerous studies worldwide have investigated the incidence of CAAs in KD, with a global average of under 1%, there is a significant gap in the literature of GiAn prevalence in the Arab world. This study aims to evaluate prevalence of GiAn in Arab countries and examine potential contributing factors in the light of global literature.

Methods: Literature review was conducted to analyze the published series across multiple Arab healthcare centers, spanning 15 countries. A total of 44 studies published between 1990 and 2023 were included, (23 case reports & 21 institutional series). Clinical manifestations and treatment with emphasis on the presence and size of CAA were collected. Relative prevalence of GiAn was calculated accordingly.

Results: The combined institutional series (n=21) reported 215 CAA among a total of 1034 KD cases (mean prevalence 20.8% [range 0-97%]). Of those, 23 CAA were GiAn (prevalence 2.22% [range 0-15%]). GiAn size ranged between 8mm and 25mm.  The rate of initial KD misdiagnosis was 4% (range 0-32%), and the average proportion of incomplete manifestations was low at 9% (range 0-72%). Interestingly, in 9/21 series incomplete KD cases were not present, with significantly higher prevalence of CAA (p=0.07), but not for GiAn (p-value=0.75). There was significant variability on IVIG treatment; on average 73% (range 10%-100%) received IVIG, including 139 cases (16%) receiving IVIG after 10 days of fever. Remarkably, the recorded lengthiest average fever duration corresponded to the series with the highest prevalence of GiAn, where fever duration 7.8 days (range 5.5-11.8 days) emerged as a pivotal factor for GiAn, (OR 5.33, 95%CI 1.1-25.77).

Conclusion: There is a higher prevalence of GiAn in the Arab countries (4.35%) compared to the global average (0.5%-1%). Initial misdiagnosis, untreated cases, delayed diagnosis and underreporting of incomplete KD are contributing factors. Low reported incomplete KD is indicative of potentially missed diagnoses and an underestimated epidemiology in those countries. The observations underscore the urgency to optimize early and proper diagnosis proficiency, highlighting important role of education of first line providers and the public, which are cornerstone of the mission of the Kawasaki Disease Arab Initiative (KAWARABI). Our findings address actionable knowledge gap and lay the foundations for targeted efforts within KAWARABI to improve outcomes for KD patients in the region.