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.
Abbreviations:
CT (computed tomography), IgG4-RD (Immunoglobulin G4-related disease), LCx (left circumflex artery), PET (positron emission tomography), TTE (transthoracic echocardiography)Keywords
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Article info
Publication history
Published online: February 22, 2023
Accepted:
February 1,
2023
Received in revised form:
January 26,
2023
Received:
January 8,
2023
Publication stage
In Press Corrected ProofIdentification
Copyright
© 2023 Japanese College of Cardiology. Published by Elsevier Ltd.
