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Immunoglobulin G4-related disease (IgG4-RD) can affect the aorta, presenting as aneurysms. Diagnosis in this case relied on IgG4 immunostaining, crucial due to normal serum levels.

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  • Immunoglobulin G4-related disease (IgG4-RD) is a fibro-inflammatory condition of unknown cause affecting multiple organs.
  • Cardiovascular manifestations, including aortic aneurysms, are rare but recognized features of IgG4-RD.
  • Diagnosis typically relies on histological findings, as serum IgG4 levels can be normal in a significant portion of patients.