CTICasesIgG4-Related Aortitis
Imaging findings
Contrast-enhanced CT and PET/CT demonstrate severe, circumferential soft-tissue thickening and wall enhancement of the thoracic aorta (aortitis) that extends down into the abdominal aorta, as well as soft-tissue encasement of the coronary arteries (specifically the right coronary artery). Multiple well-defined mediastinal and hilar lymph nodes with high FDG avidity are present. Pathologic biopsy of a groin lymph node and kidney confirms IgG4-related disease.
Key takeaways
IgG4-related disease is a systemic fibroinflammatory condition that can affect multiple organ systems. When it involves the cardiovascular system, it typically manifests as aortitis or periaortitis (often mimicking retroperitoneal fibrosis or large vessel vasculitis like Takayasu's arteritis) and can involve the coronary arteries or cause renal disease. Pathologic biopsy showing IgG4-positive plasma cell infiltration is diagnostic, and patients usually respond well to steroids.
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