Coronary artery abnormalities in children with systemic-onset juvenile idiopathic arthritis
Alain Lefèvre-Utile1, Caroline Galeotti1, Isabelle Koné-Paut1
1Department 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.
Insights
Systemic-onset Juvenile Idiopathic Arthritis (SoJIA), or Still's disease, can present with coronary artery abnormalities, challenging its diagnosis. These findings do not exclude Still's disease and may lead to misdiagnosis as Kawasaki disease.
Area of Science:
- Pediatrics
- Rheumatology
- Cardiology
Background:
- Systemic-onset Juvenile Idiopathic Arthritis (SoJIA), or Still's disease, is characterized by fever, rash, and arthritis.
- Diagnosis can be challenging, with conditions like Kawasaki disease often presenting similarly.
- Coronary artery abnormalities are typically associated with Kawasaki disease, aiding differentiation.
Purpose of the Study:
- To highlight cases of Still's disease misdiagnosed as Kawasaki disease due to coronary artery abnormalities.
- To emphasize that coronary artery abnormalities do not exclude a diagnosis of Still's disease.
Main Methods:
- Case series presentation of four children with Still's disease and coronary artery abnormalities.
- Review of clinical presentation, echocardiographic findings, and treatment responses.
- Differential diagnosis considerations between Still's disease and Kawasaki disease.
Main Results:
- Four patients with Still's disease initially misdiagnosed as Kawasaki disease were identified.
- Observed coronary artery abnormalities included irregular walls, dilation, thickened walls, and hyperechogenicity.
- Intravenous immunoglobulin (IVIG) treatment was ineffective, and persistent arthritis confirmed the SoJIA diagnosis.
- Most patients required biologic treatments for Still's disease.
Conclusions:
- Coronary artery abnormalities can occur in Still's disease and should not rule out this diagnosis.
- Clinical vigilance is crucial for differentiating Still's disease from Kawasaki disease, especially when cardiac findings are present.
- Further research is needed to understand the spectrum of cardiac manifestations in SoJIA.
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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