RESUMEN
Immunoglobulin G4 (IgG4)<bk wid=1q><bm>-related disease can occur in various organs, most of which comprise glandular or ductal tissue. We report a case of IgG4-related disease which occurred in a cardiovascular lesion. A 69-year-old man was found to have a tumorous lesion around the coronary artery. Open chest biopsy showed the diffuse lymphoplasmacytic infiltration, occasional eosinophils and numerous IgG4-positive plasma cells within the lesions. The serum concentration of IgG4 in the postoperative period was 1,080 mg/dl (reference range, <135). We diagnosed IgG4-related periarteritis manifesting as a tumor around the coronary artery. This case suggests that IgG4-related disease can occur around the coronary artery and manifest as a periarterial mass lesion.