Diagnostic Imaging Studies
Clinical diagnosis of sJIA-LD was supported by chest computed tomography (CT) for all patients. Representative images from the initial diagnostic image are shown in Figure 1 for all nine patients. The most common findings were septal thickening (n=9), ground glass opacities (n=8), and peripheral consolidation (n=5). Other reported findings included adenopathy, interstitial opacities, subsegmental mosaic attenuation, mild bronchiectasis, and findings suggestive of pulmonary fibrosis (Table 3). One patient had findings consistent with larger pulmonary artery concerning for possible pulmonary hypertension. The lower lobes were most frequently affected.
In five patients, serial CT imaging was obtained to guide management (Figure 2). For patient 1, chest imaging showed improvement after intensive treatment with monthly IV methylprednisolone, etoposide, IVIG and tocilizumab; subsequently this patient was able to be weaned to IVIG and tocilizumab alone. For patient 4, chest imaging showed clinical progression of sJIA-LD despite intensive treatment with failure of multiple immunosuppressive medications. This was subsequently stabilized after bone marrow transplantation for refractory sJIA. Despite similar chest imaging before and after bone marrow transplantation, patient 4 did have reversal of spirometry findings. Patient 5 showed improvement in chest imaging after treatment with tocilizumab and tofacitinib. Imaging for patient 6 reflects initial worsening fibrosis, which followed COVID infection, admission for MAS, and prolonged cough. Subsequent imaging showed stable changes following increased rheumatologic and pulmonology treatment, including tocilizumab, methylprednisolone, tofacitinib, fluticasone propionate / salmeterol, azithromycin, and montelukast. For patient 9, the initial presentation was concerning for MAS, and chest CT findings reflect mainly ground glass opacities and pleural effusions. Subsequent images lead to diagnosis of sJIA-LD as they were more consistent with septal thickening, peripheral consolidation, and bronchial thickening. Patient 9’s imaging also stabilized after treatment with methylprednisolone, tocilizumab and tofacitinib.
Eight patients completed at least one echocardiogram, with the most recent results within normal limits for five cases. Significant findings include left ventricular diastolic dysfunction for one case, mild left ventricular dilation for one case, and mild right coronary artery and left anterior descending coronary artery dilation for one case.