THU0508 LARGE VESSEL VASCULITIS IN A COHORT OF CHILDREN WITH RESISTANT KAWASAKI DISEASE IN SINGAPORE
Authors: L. Das, J. H. T. Tan, T. Arkachaisri
Journal: Annals of the Rheumatic Diseases (2020)

Background: Kawasaki Disease (KD) is one of the most common systemic vasculitidies in children today. IVIG is the mainstay of treatment, however, about 1/5 of patients do not respond resulting in an increased risk of Coronary Artery Abnormalities (CAA) 1 . Objectives: To describe a cohort of infants and young children with resistant Kawasaki Disease (rKD) who were noted to have prolonged difficult courses with resultant CAA and ultimately diagnosed with Aortitis. Methods: Demographic, clinical manifestations and imaging data were collected from children seen in Rheumatology clinic with a diagnosis of rKD and aortitis. Nonparametric descriptive statistics were used to describe the data. Results: Between 2010-2018, 63 out of 417 KD referrals were diagnosed with rKD. 7 children had prolonged time to CRP normalisation, prolonged admission or recurrence of symptoms (Table 2). All patients underwent Magnetic Resonance Angiography (MRA) and were found to have evidence of large vessel arteritis consisting of wall irregularity, thickness and contrast enhancement. Table 1 ID Age at diagnosis (mo) Number of clinical KD signs** BCG site inflammation Lowest Hemoglobin g/DL Highest WBC 10 (9)L #Highest Platelet 10 (9)L Highest CRP mg/L Lowest Albumin g/L Maximum ALT U/L Maximum AST U/L A 31 3 0 NA NA NA NA NA NA NA B 7 4 0 9.5 37.3 875.0 206.0 21.0 36.0 32.0 C 4 4 1 9.8 43.2 918.0 130.0 22.0 44.0 52.0 D 3.5 4 1 9.2 42.4 1715.0 181.4 18.0 61.0 99.0 E 5 4 1 6.7 17.6 1413.0 148.2 27.0 20.0 24.0 F 2 3 0 6.4 35.6 959.0 212.1 15.0 8.0 19.0 G 24 NA NA 8.6 21.9 1002.0 181.4 27.0 17.0 30.0 5 patients were male; All were Singaporean Chinese except for G who was from the Philippines; A &amp; G were treated in an outside facility/separate service initially and some data was not available; # Blood test results were generally taken at the same time apart from the platelet count; **Only D presented with lymphadenitis. Table 2 ID TIme to CRP normalization (weeks) Number of IVIG given Steroids Time to abnormal echo (weeks) Time to aneurysm development (weeks) Other outcomes A * 2 oral only 4 4 1 st IVIG given 3 weeks after symptom onset B * 2 IV/PO 2 2 deceased, MI secondary to thrombus C 2 1 IV/PO 1.1 0 treated with Infliximab and then Methotrexate, post infliximab psoriasis, D 5 1 IV/PO 0.3 2 treated with Infliximab and then Methotrexate, multiple infections in hospital; BCG reactivation while on Infliximab E 9 2 no 1.4 2.7 UTI, AIHA, lost to follow up F 3 3 IV/PO 0.71 2.5 post Infliximab psoriasis, multiple infections in hospital G 7 2 oral only 7 0 lost to follow up *patients A &amp; B had recurrent KD episodes interspersed with short periods of symptom resolution Conclusion: While transthoracic echocardiograms remain imaging investigation of choice, for children with recalcitrant or recrudescent KD, especially in the setting of delayed CRP resolution of &gt;2 weeks; an MRA should be considered. References: [1]McCrindle BW, Rowley AH, Newburger JW, et al. Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association. Circulation. 2017 Apr 25;135(17):e927-e999. Disclosure of Interests: None declared