CTICasesIgG4-Related Disease
Imaging findings
Frontal chest radiograph and non-contrast CT show a large, mass-like consolidation in the right upper lobe mimicking infectious pneumonia, which enlarged over a two-and-a-half-week period despite antibiotic therapy. FDG-PET/CT reveals prominent metabolic activity in the right upper lobe consolidation and extensive, hypermetabolic soft tissue encasing the retroperitoneal aorta and iliac arteries, pulling them inward.
Key takeaways
IgG4-related disease is a systemic fibroinflammatory condition that can manifest in the lungs as pseudotumoral consolidations, nodules, or interstitial disease. Histopathologically, it is characterized by dense lymphoplasmacytic infiltrates rich in IgG4-positive plasma cells, storiform fibrosis, and obliterative phlebitis. Multi-organ involvement, such as concurrent retroperitoneal fibrosis and pulmonary mass-like consolidation, is highly suggestive of the diagnosis and typically responds well to corticosteroid therapy.
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