CTICasesIgG4-Related Disease with Lung Parenchymal and Biliary Involvement
Imaging findings
Historical CT images from 2005 show a soft-tissue mass at the hepatic flexure. Subsequent imaging shows persistent biliary strictures requiring multiple stent placements. Recent CT and PET/CT demonstrate new multifocal, mass-like consolidations in the bilateral lungs with associated hypermetabolic activity, as well as hypermetabolic paraspinal soft tissue and mediastinal/internal mammary lymphadenopathy. Follow-up imaging after high-dose steroid therapy shows substantial improvement of the lung parenchymal opacities.
Key takeaways
IgG4-related disease is a systemic fibroinflammatory condition that can mimic malignant tumors or systemic vasculitis across multiple organs. Pathologically, it was previously misdiagnosed in this patient as a spindle cell neoplasm or inflammatory myofibroblastic tumor. In the lungs, IgG4-related disease can present with mass-like consolidations, nodular opacities, or bronchovascular thickening, often responding dramatically to corticosteroid therapy.
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