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IgG4-Related Sclerosing Disease

IgG4-Related Sclerosing Disease▶ Watch on YouTube — age-restricted, cannot embed here

Imaging findings

CT scans demonstrate ill-defined, non-segmental parenchymal opacities in the bilateral lower lobes, accompanied by distinct extrapleural, paravertebral soft tissue masses. Associated systemic findings include a mass-like enlargement of the pancreatic tail, biliary tree involvement causing cholangitis, a right submandibular gland mass consistent with a Küttner tumor, and infiltrative renal lesions.

Key takeaways

IgG4-related disease is a systemic fibroinflammatory condition that can present with a wide array of thoracic findings, including lung nodules, interstitial patterns, and extrapleural paravertebral masses. Recognizing multi-organ involvement, particularly in the salivary glands, pancreas, biliary tract, and kidneys, is critical to arriving at the diagnosis, which is supported by elevated serum IgG4.

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