Abstract
Immunoglobulin G4-related disease (IgG4-RD) of the cardiovascular system is rare. Multiple approaches for the management of IgG4-RD have been reported, including surgical resection of affected tissues and treatment with systemic glucocorticoids being generally used. Therefore, the results of surgical resection alone are unknown. A 79-year-old male underwent total aortic arch replacement 5 years previously. Subsequently, a coronary aneurysm of the left circumflex artery (LCx) enlarged with pericardial effusion was removed 2 years after the first surgery. He was diagnosed with a confirmed IgG4-related coronary aneurysm. Serum IgG4 level was 331 mg/dL, and the aneurysm at the distal portion of the LCx was residual. However, he did not receive any corticosteroid treatment. Follow-up transthoracic echocardiography (TTE) revealed an abnormal echo-free cavity structure at 5 o'clock position of the short-axis view. This case describes the progression of residual IgG4-related coronary aneurysm, without corticosteroid therapy. A case with thoracic aortic disease combined with coronary aneurysm may be IgG4-RD. The possibility of progression is high in cases where serum IgG4 remains at a high level, especially without steroid treatment, and follow-up examinations, such as TTE and computed tomography, are more important. Therefore, we reaffirm that corticosteroid therapy may have been important. LEARNING OBJECTIVE: Immunoglobulin G4-related disease (IgG4-RD) of the cardiovascular system is rare. Multiple approaches for the management of IgG4-RD have been reported, including surgical resection of affected tissues and treatment with systemic glucocorticoids being generally used. Therefore, the results of surgical resection alone, to avoid steroid-related complications, are unknown. Our case showed thoracic aortic disease combined with coronary aneurysm may be IgG4-RD. The importance of corticosteroid treatment was reaffirmed because the residual coronary aneurysm progressed without corticosteroid treatment.