case_images: PMC10070724_01
case_images: (A) Chest x-ray showed significant cardiomegaly. (B-E) MPR images of thoracic CTA. (B) Sagittal view of CTA demonstrated stenosis of the descending aorta and upper abdominal aorta with thickening of the vascular wall. (C) Axial view of CTA showed that the descending aorta was stenosed to 3.1 mm. (D,E) Sagittal oblique view and axial view of CTA indicated complete occlusion of the descending aorta with large thrombus (76 mm in length). (F,G) MIP image and VR reconstruction of CTA revealed complete occlusion of the descending aorta with large thrombus (76 mm in length) and extensive collateral circulation (twisted and dilated right intrathoracic artery, numerous open collateral vessels in the neck and upper mediastinum). (H) Axial view of brain MR imaging revealed high signals on T2 FLAIR images of cortical and subcortical areas of both the occipital lobes and right frontal-parietal lobe. MPR, multiplanar reconstruction; CTA, computed tomography angiography; MIP, maximum intensity projection; VR, volume rendered; MR, magnetic resonance; FLAIR, fluid-attenuation inversion recovery.
case_images: fcvm-10-1076118-g001.jpg
case_images: PMC10070724_01_fcvm-10-1076118-g001.jpg
case_images: F1
case_images: Chest x-ray showed significant cardiomegaly (Figure 1A). CT showed calcified left hilar lymph nodes, enlarged mediastinal lymph nodes, patchy and slightly high-density shadows in both lungs, and a small amount of bilateral pleural effusion. CT angiography (CTA) showed occlusion of the proximal left subclavian artery and stenosis of the lumen of the descending and upper abdominal aorta with extensive thickening of the vascular wall (Figures 1B,C). Repeated CTA on day 77 and day 92 showed complete occlusion of the descending aorta with large thrombus (76 mm in length) (Figures 1D,E) and extensive collateral circulation (twisted and dilated right intrathoracic artery, numerous open collateral vessels in the neck and upper mediastinum) (Figures 1F,G). Brain MR imaging revealed strong signals on T2 fluid-attenuation inversion recovery (FLAIR) images of the cortical and subcortical areas of both the occipital lobes and right frontal-parietal lobe (Figure 1H).
case_images: Clinical course of this case and sequential changes in NT-proBNP and CRP. NT-proBNP, N-terminal pro-brain natriuretic peptide; CRP, C-reactive protein; IVMP, intravascular methylprednisolone pulse; TCZ, tocilizumab.
case_images: fcvm-10-1076118-g002.jpg
case_images: PMC10070724_01_fcvm-10-1076118-g002.jpg
case_images: F2
case_images: On day 32, intravenous tocilizumab (TCZ) was initiated at a dose of 8 mg/kg following the 0-, 2-, and 4-week regimen (Figure 2). The patient died on day 133 (Figure 2).