Clinical analysis of 5 cases of systemic juvenile idiopathic arthritis with dilation of the coronary arteries


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Zhonghua Er Ke Za Zhi. 2022 May 2;60(5):462-465. doi: 10.3760/cma.j.cn112140-20210923-00818.


Goal: To study the clinical features of systemic juvenile idiopathic arthritis associated with coronary artery dilation. Methods : A retrospective analysis was performed on the clinical data including clinical manifestations, blood routine, inflammatory factors, echocardiography, vascular ultrasound and CT angiography, treatment and outcome, etc., of 5 cases with systemic juvenile idiopathic arthritis associated with coronary artery dilation admitted to the Rheumatology Department at the Affiliated Children’s Hospital of the Capital Institute of Pediatrics from May 2019 to June 2021. Results: There were 2 men and 3 women among 5 cases. Age at onset ranged from 7 months to 4 years and 7 months. Time to diagnosis ranged from 1.5 months to 3.0 months. Four cases were diagnosed as atypical Kawasaki disease. Three cases showed unilateral dilation of the coronary artery. Two cases showed bilateral dilation of the coronary artery. Four cases developed multiple organ lesions. Three cases developed macrophage activation syndrome. Three cases developed lung injury. treatment, 3 cases treated with pulse therapy of methylprednisolone and methotrexate combined with cyclosporine, improved after the last application of biological agents, and stopped prednisone. The other 2 cases were treated with adequate and gradually reduced oral prednisone, and methotrexate was added at the same time, 1 case relapsed in the reduction process. No other vascular involvement was found in 5 cases. Coronary artery dilation fully recovered after 1-3 months of treatment. Conclusion : Systemic juvenile idiopathic arthritis associated with coronary artery dilation has the clinical characteristics of early onset age, long duration of diagnosis, prone to multiple organ damage. Conventional corticosteroids and immunosuppressive agents are insensitive and biological agents should be used as soon as possible. The prognosis for coronary artery dilation is good after prompt treatment.

PMID:35488642 | DOI: 10.3760/cma.j.cn112140-20210923-00818

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