title: Coronary artery abnormalities in children with systemic-onset juvenile idiopathic arthritis.
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.
authors.affiliation: Department of Paediatrics, Paediatric Rheumatology, National referral centre of auto-inflammatory diseases, CEREMAI, CHU de Bicêtre, AP-HP, University of Paris SUD, 94275 Le Kremlin-Bicêtre, France.
authors.forename: Alain
authors.initials: A
authors.lastname: Lefèvre-Utile
authors.affiliation: Department of Paediatrics, Paediatric Rheumatology, National referral centre of auto-inflammatory diseases, CEREMAI, CHU de Bicêtre, AP-HP, University of Paris SUD, 94275 Le Kremlin-Bicêtre, France. Electronic address: caroline.galeotti@bct.aphp.fr.
authors.forename: Caroline
authors.initials: C
authors.lastname: Galeotti
authors.affiliation: Department of Paediatrics, Paediatric Rheumatology, National referral centre of auto-inflammatory diseases, CEREMAI, CHU de Bicêtre, AP-HP, University of Paris SUD, 94275 Le Kremlin-Bicêtre, France.
authors.forename: Isabelle
authors.initials: I
authors.lastname: Koné-Paut
mesh_terms: D000293:Adolescent; D001171:Arthritis, Juvenile / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D002675:Child, Preschool; D003324:Coronary Artery Disease / Q000000981:diagnostic imaging* / Q000209:etiology / Q000628:therapy; D003937:Diagnosis, Differential; D003951:Diagnostic Errors; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009080:Mucocutaneous Lymph Node Syndrome / Q000175:diagnosis; D012189:Retrospective Studies; D014463:Ultrasonography
keywords: Cardiac echography; Coronary dilatation; Kawasaki disease; Still disease; Systemic-onset Juvenile Idiopathic Arthritis