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.