PMC6082998_01
MR imaging of the abdomen in IgG4-related disease.. A) Magnetic resonance cholangiopancreatography (MRCP) shows interruption of the dilated pancreatic duct (arrow) and the common bile duct (hollow arrow) at the pancreatic head (double duct sign).. B) Coronal T2-weighted MR image shows a pancreatic head lesion (arrow) obstructing the common bile duct (hollow arrow).. C) Coronal T2-weighted MR image shows multiple bilateral renal lesions.. D) Transverse steady-state free precession MR image shows thickening of the wall of the abdominal aorta (arrow).
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MRI/MRCP (Fig. 1) revealed dilated biliary and pancreatic ducts (double duct sign) with a pancreatic head mass, multiple wedge-shaped lesions in both kidneys, significant aortic thickening suggestive of aortitis/Peri-aortitis, and cervical, mediastinal and intra-abdominal lymph node enlargement.
FDG PET/CT: FDG PET/CT maximum intensity projection (upper row) and fused trans-axial images (lower row) showing multiple lymph nodal involvement, lacrimal/salivary gland uptake, a right pulmonary focus, a hypermetabolic pancreatic head lesion, renal cortical lesions, and intense circumferential uptake in the abdominal aorta.
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An FDG PET/CT scan (Fig. 2) showed intense circumferential uptake in the abdominal aorta corresponding to the MRI findings, and suggestive of aortitis.
Histopathology.. A) Low power examination of the lymph node showed a lymphoid follicle with a reactive germinal center and part of an expanded paracortical area with dense infiltrate.. B) High power examination showed a marked plasma cells infiltrate.. C) Immunohistochemical stain for CD138 highlighted many plasma cells, some of which are seen inside the germinal center.. D) IgG4 Immunohistochemical stain revealed more than 30 cells seen per high power field.
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The overall morphology and Immunohistochemistry were consistent with IgG4-related disease (Fig. 3).