Coronary artery abnormalities in children with systemic-onset juvenile idiopathic arthritis |
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Institution: | 1. Medical Research Center of Shandong Provincial Qianfoshan Hospital, Shandong University, Jingshi road 16766, Jinan, Shandong, 250014 P.R. China;2. Rheumatology Department of Shandong Provincial Hospital, Jinan, Shandong, 250014 P.R. China;1. Division of Human Genetics,;2. Division of Bone Marrow Transplantation and Immune Deficiency, Cancer and Blood Diseases Institute, and;3. Division of Developmental Biology, Cincinnati Children''s Hospital Medical Center, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH;1. Unidad de Reumatología Pediátrica, Servicio de Reumatología, Hospital Universitario Ramón y Cajal, Madrid, España;2. Servicio de Anatomía Patológica, Hospital Universitario Ramón y Cajal, Madrid, España |
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Abstract: | Still's disease (Systemic-onset Juvenile Idiopathic Arthritis: SoJIA) is characterised by high-spiking daily fevers, arthritis and evanescent rashes. Diagnosis of Still's disease is often challenging. Infectious diseases and other inflammatory conditions, especially in young children, Kawasaki disease may look similar. Clinicians often rely on echocardiographic evidence of coronary artery abnormalities to differentiate between Kawasaki disease and Still's disease. Coronary artery dilation would typically favour the diagnosis of Kawasaki disease. We present four children with Still's disease and coronary artery abnormalities who were initially misdiagnosed as Kawasaki disease. The first patient had pericarditis and an irregular wall of the left coronary artery, without dilation on echocardiography. The second patient had a left coronary artery dilatation and a pericarditis. The third patient had thickened left coronary artery walls, and the fourth patient had a hyperechogenicity of the left and right coronary arteries. They received IVIG without success. The diagnosis of Still's disease was made secondary with evidence of persistent arthritis. All but one patient finally needed biologic treatments. Coronary abnormalities may be observed during various febrile conditions and do not exclude the diagnosis of Still's disease. |
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Keywords: | Systemic-onset Juvenile Idiopathic Arthritis Still disease Kawasaki disease Coronary dilatation Cardiac echography |
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