CTICasesIgG4-Related Sclerosing Disease of the Lung
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Imaging findings
Chest x-ray showed a mass and multiple nodules. CT showed a large mass with air bronchograms, other nodules, and new areas of peribronchovascular ground-glass opacities. Some nodules enlarged over time. Imaging findings were noted to be non-specific, resembling adenocarcinoma or various interstitial lung diseases.
Key takeaways
This patient had a history of multiple sclerosis treated with interferon beta, developed a non-productive cough, and was diagnosed with IgG4-related sclerosing disease of the lungs after biopsy showed plasma cells, follicular bronchiolitis, ionizing sclerosis, and positive IgG4 stains. Stopping interferon beta treatment initially improved symptoms, but the disease later recurred, requiring prednisone. Lung involvement in IgG4-related disease can manifest as nodules, masses, ground-glass opacity, bronchovascular consolidation, and sometimes honeycombing or fibrosis. Although often associated with autoimmune pancreatitis, this patient did not have other organ involvement.
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