PMC6582838_01
CT scan when the patient was first diagnosed with IgG4-RD with peritoneal fibrosis and periaortitis. CT, computed tomography; IgG4-RD, IgG4-related disease.
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He had been diagnosed with peritoneal fibrosis, periaortitis, and bilateral parotitis based on a computed tomography (CT) scan (Figure 1) and physical examination findings.
Pathology of the parotid gland at the diagnosis of IgG4-RD. (a) IgG staining and (b) IgG4 staining. There are many plasma cells stained with IgG (a) and 90% of them were positive for IgG4 (b). IgG4-RD, IgG4-related disease; IgG, immunoglobulin G.
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The serum level of IgG4 was 3260 mg/dl, and biopsy of the parotid gland showed infiltration of IgG4-positive plasma cells (Figure 2).
CT scan when the patient first came to our hospital. (a) The abdominal aorta was enlarged, and the adventitia was thickened. (b) Soft tissues around the ureters were thickened. (c) The tissue around the bilateral internal iliac arteries was thickened. (d) The adventitia of the bilateral femoral arteries was thickened and lymph nodes were swollen. CT, computed tomography; FA, femoral artery; LN, lymph node.
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A CT scan showed a 44 mm diameter AAA with thickened adventitia (Figure 3(a)). The CT scan also showed thickening of tissue around the ureters (Figure 3(b)), internal iliac arteries (Figure 3(c)), and femoral arteries (Figure 3(d)). The lymph nodes were also swollen (Figure 3(d)).
T2-weighted MRI at the T12 level showed bilateral hyperintensities in the anterior horns of the grey matter. MRI, magnetic resonance imaging.
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Based on magnetic resonance imaging (MRI) findings (Figure 4), the neurologist concluded that he had paraplegia from the 11th thoracic spinal cord level due to anterior spinal artery syndrome.
Coronary angiogram at the time of myocardial infarction (a) and after endovascular intervention (b). Angiogram at the time of myocardial infarction (a) showing occlusion of the left anterior descending artery with thrombus.
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Aspiration of the thrombus and stenting for the occluded left descending artery were performed (Figure 5).
Progress of manifestations, IgG, IgG4, and treatments. IgG and IgG4 serum levels became normal 1 month after PSL administration. PSL, prednisolone; IgG, immunoglobulin G.
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Autopsy revealed wide range of intestinal erosion with ischemia (Figure 6).