Background Systemic-onset juvenile idiopathic arthritis (SoJIA) is characterized by hectic daily fevers, arthritis and evanescent rashes. Diagnosis of SoJIA is often challenging. Infectious diseases and other inflammatory conditions, including Kawasaki disease (KD) may mimic SoJIA. By some way SoJIA and KD share common pattern of autoinflammatory diseases such as fever, rash, and marked elevation of acute phase reactants. Clinicians often rely on echocardiographic evidence of coronary artery dilation to differentiate KD and SoJIA, especially when KD feature is uncomplete. Coronary artery dilation would typically favor the diagnosis of KD. However, in a prospective study in 2004, 5 patients among 12 with SoJIA had coronary artery dilation 1.
Objectives We present three other children with SoJIA, and coronary artery abnormalities at the beginning of their disease.
Methods Patients had an echocardiography before the diagnosis of SoJIA, and while we suspected KD.
Results The diagnosis of SoJIA was made at the age of 10 months for two patients and 15 months for one patient. KD was suspected initially because they were toddlers with prolonged fever, cutaneous rash and marked biological inflammation. The initial echocardiography of the first patient showed pericarditis and irregular wall of the left coronary artery, without dilation. The second patient had left coronary artery dilation and pericarditis. The third patient had thickening of the left coronary artery wall, without dilatation or pericarditis. They both received IVIg without success. SoJIA was recognized because they did not respond to two infusions of IVIg and because they subsequently developed arthritis. The three patients evolved toward severe corticodependant SoJIA responsive to tocilizumab only in two of them due to refractory polyarthritis.
Conclusions In very young patients, KD and SoJIA may be confused at the beginning not only by similar observable clinical symptoms but also by the eventuality of subclinical and transient coronary arteries vasculitis. The association of coronary abnormalities to higher severity of SoJIA needs to be confirmed.
Binstadt BA, Levine JC, Nigrovic PA, et al. Coronary artery dilation among patients presenting with systemic-onset juvenile idiopathic arthritis. Pediatrics 2005; 116:e89-93.
Disclosure of Interest None Declared