CTICasesIgG4-Related Disease
Imaging findings
Serial chest CT scans show progressive, bilateral pleural thickening and loculated pleural effusions, accompanied by a large infiltrating soft-tissue mass within the anterior mediastinum. The mediastinal process directly abuts and cuffs the ascending thoracic aorta, compressing it into an ovoid shape. Mediastinal biopsy shows dense polyclonal plasma cell infiltration with 20% IgG4-positive plasma cells on immunoperoxidase staining, without lymphoma.
Key takeaways
IgG4-related disease is a systemic fibroinflammatory condition characterized by tissue infiltration with IgG4-positive plasma cells, storiform fibrosis, and obliterative phlebitis. In the thorax, it can present as fibrosing mediastinitis, pseudotumor, pleural thickening, or aortitis/periaortitis. Because it is highly responsive to corticosteroids, differentiating it from lymphoma, mesothelioma, or other fibrosing disorders is critical.
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