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IgG4-related lung disease

Imaging findings

Chest CT shows multifocal, well-demarcated nodular and consolidative lung opacities with surrounding ground-glass halos, which demonstrate a waxing and waning course on serial imaging. Biopsies of a cervical lymph node and the lung lesion showed a lymphoplasmacytic infiltrate with increased IgG4-positive plasma cells.

Key takeaways

IgG4-related lung disease is a systemic fibroinflammatory disorder that can present as progressive, multifocal, mass-like consolidations or nodules in the lungs that wax and wane. It mimics lymphoma or granulomatous disease and typically responds well to steroid therapy.

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