CTICasesRetroperitoneal Fibrosis and Pulmonary Fibrosis (IgG4-Related Disease)
Imaging findings
Abdominal CT demonstrates extensive retroperitoneal fibrous tissue coating the aorta, iliac arteries, and ureters, causing right kidney atrophy. Chest CT shows a stable, well-defined rind of peripheral pulmonary scarring with air bronchograms along the left chest wall, stable over decades.
Key takeaways
Idiopathic retroperitoneal fibrosis is a major manifestation of IgG4-related disease. IgG4-related disease is a systemic fibroinflammatory disorder that can affect almost any organ system, including the lungs where it can present with atypical, well-demarcated rinds of subpleural/parenchymal scarring that remain stable over long periods.
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