Background Large vessel involvement following recrudescent or recalcitrant Kawasaki Disease or other vasculitidies in young children have been limited to few case reports and outcomes are still unclear.
Objectives To describe and compare the characteristics and outcomes of 6 patients with large vessel vasculitis diagnosed between 2013 to 2015 in KK Hospital, Singapore
Methods Demographic and disease characteristic information were collected and median, interquartile range (IQR) & percentiles were used to describe the data.
Results 6 patients were included in the analysis. Median age was 3.75 months (IQR 3.75, 4). Other than 1 Filipino 2 year old girl (patient C), all other infants were of Chinese Singaporean origin and were less than age 1 year at onset; 4 male and 1 female. All patients were found to have multiple areas of irregular, thickened vessel walls with enhancement, involving the aorta and additional medium sized arteries on Magnetic Resonance Angiography (MRA).
Only patients C and F presented with prolonged fever and or less than 1 clinical feature of KD. C developed a unilateral enlarged lymph node on recrudescence of fever 1 month after the initial event. She was then found to have dilated CA (dCA) and consequently abnormal MRA. Other patients had significant KD Index scores and complete KD presentations.
Of the 4 patients who received only 1 dose of IVIG; C and F had diagnosis change from atypical KD to Takayasu Arteritis and G developed rhinovirus and then rotavirus as reason for prolonged fever. Patient A received IV steroids and then Infliximab. All patients received steroids. Patients A and B both developed a psoriasiform rash post-Infliximab which resolved on follow up.
Patient F and Patient B died 3 months and 17 months after diagnosis. Patient F, presented with fever, fussiness and enteritis aged 4 months. Her symptoms recurred 2 weeks after onset. Due to differential hypertension, she received an echocardiogram and subsequently MRA and cardiac catherization. Cause of death at age 7 months was small bowel perforation with arteritis and consequent intra-abdominal sepsis.
Patient B, presented with complete features of KD at age 2 months. His course was noted for recrudescence 2 weeks and 4 weeks after initial IVIG. He developed giant coronary aneurysms with abnormal MRA findings; developed myocardial infarction at age 8 months and proceeded to require a Left Ventricular Assist Device but died age 13 months from multi-organ failure.
3/6 (50%) developed aneurysms, D and F were giant. 4 surviving patients, had improvement in all vessels but continued activity in the aorta on follow up MRA, they remain on Methotrexate with no active clinical findings.
Conclusions Extensive involvement of the systemic arteries and aorta in all patient were noted on initial MRA. Follow up imaging, showed improvement in all vessels. After 1 year of follow up, complete resolution occurred only in medium vessels.
Mortality in this patient cohort was 2/7 (28.6%).
This is an observational case series and our findings will need to be reproduced in larger groups of patients.
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McCulloch M, Andronikou S, Goddard E, et al. Angiographic features of 26 children with Takayasu's arteritis. Pediatr Radiol (2003) 33: 230–235.
Disclosure of Interest None declared