| Summary: | Immunoglobulin G4-related disease (IgG4-RD) is a chronic inflammatory condition involving
multiple organs, including the salivary or lacrimal glands, orbit, pancreas, bile duct, liver, kidney,
retroperitoneum, aorta, lung, and lymph nodes. It is histologically characterized by tissue
infiltration with lymphocytes and IgG4-secreting plasma cells, storiform fibrosis, and obliterative
phlebitis. In the thoracic involvement of IgG4-RD, mediastinal lymphadenopathy and perilymphangitic
interstitial thickening of the lung are the most common findings. Peribronchovascular
and septal thickening and paravertebral band-like soft tissue are characteristic findings of
IgG4-RD. Other findings include pulmonary nodules or masses, ground-glass opacity, alveolar
interstitial thickening, pleural effusion or thickening, mass in the chest wall or mediastinum,
and arteritis involving the aorta and coronary artery. Radiologic differential diagnosis of various
malignancies, infections, and inflammatory conditions is needed. In this review, we describe
the imaging findings of IgG4-RD and the radiologic differential diagnoses in the thorax.
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