CTICasesIgG4-Related Sclerosing Disease of the Lung
Imaging findings
Frontal radiograph shows multifocal, multilobar large nodular ill-defined opacities with associated reticulation. CT reveals extensive airway-centric disease distributed along the bronchovascular bundles and perilymphatic regions abutting interlobar fissures, with some airways narrowed, occluded, or filled with secretions despite patent airways traversing many opacities. There is admixed cystic change and modest mediastinal and intrapulmonary lymph node enlargement. Serial imaging back to 2019 shows persistent, mildly progressive disease.
Key takeaways
Pulmonary IgG4-related sclerosing disease can present in a florid, extensively airway-centric nodular form that mimics adenocarcinoma or a lymphoproliferative disorder, and lung involvement is uncommon and usually more circumscribed. Surgical lung biopsy is often required, showing the classic features of storiform fibrosis, obliterative phlebitis, and abundant IgG4-positive plasma cells. Perilymphatic and peribronchovascular distribution with mediastinal lymphadenopathy overlaps with sarcoidosis, and immunoglobulin light-chain deposition may account for some of the associated cystic change.
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