Large Progressive Right Coronary Artery Aneurysm Secondary to IgG4 Disease.

BACKGROUND

Coronary artery aneurysm is a rare but well recognized manifestation of immunoglobulin G4 (IgG4)-related disease, with a paucity of data to guide its management.

CASE SUMMARY: A 62-year-old man with known IgG4-related disease diagnosed 2 years prior presented for routine clinic follow-up. Despite being asymptomatic and remaining in serologic remission, imaging demonstrated marked progression of a large right coronary artery aneurysm measuring 62 mm × 72 mm. Given prohibitive operative risks, immunosuppressive therapy was intensified with close imaging follow-up.

DISCUSSION: Published experience with IgG4-related coronary artery aneurysm is limited to small case series, with highly variable management strategies and outcomes. To the authors' knowledge, this case represents the largest conservatively managed IgG4-related coronary aneurysm reported.

TAKE HOME MESSAGES: IgG4-related coronary aneurysms may progress silently with disassociation between serologic remission and disease progression on imaging. Multidisciplinary decision-making is critical to optimize outcomes in this high-risk condition, particularly with regard to the surveillance strategy in patients who otherwise achieve biochemical remission.