title: Giant tumorous lesions (correction of legions) surrounding the right coronary artery associated with immunoglobulin-G4-related systemic disease.
abstract: Immunoglobulin G4 (IgG4)-related systemic disease was first recognized as a clinicopathological entity about 10 years ago, and since then, it has attracted growing attention. It is an autoimmune disease which affects multiple organs including the pancreas, bile duct, salivary glands and retroperitoneum. Further, it was recently reported that it can be manifested as periarteritis, often as inflammatory abdominal aortic aneurysm. We describe the case of a 75-year-old man with autoimmune pancreatitis and parotitis who presented with angina. The serum concentration of IgG4 was significantly increased at 2,510 mg/dl. Coronary angiography showed multiple stenotic lesions and pronounced dilatation of the right coronary artery. Cardiac computed tomography disclosed increased wall thickness of the coronary arteries and focal tumorous lesions surrounding the right coronary artery. Treatment with steroids proved only marginally effective and he underwent surgical resection of the aneurysm and coronary artery bypass grafting. The diagnosis of IgG4-related systemic disease was confirmed by histological examination of the resected mass, which showed a massive infiltration of IgG4-positive plasma cells. This case emphasizes the importance of considering the diagnosis in any patient with abnormally increased wall thickness or ectatic lesions in the coronary arteries.
authors.affiliation: Department of Cardiovascular Medicine, The University of Tokyo, Tokyo, Japan.
authors.forename: Masayasu
authors.initials: M
authors.lastname: Ikutomi
authors.forename: Takayoshi
authors.initials: T
authors.lastname: Matsumura
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Iwata
authors.forename: Go
authors.initials: G
authors.lastname: Nishimura
authors.forename: Nobukazu
authors.initials: N
authors.lastname: Ishizaka
authors.forename: Yasunobu
authors.initials: Y
authors.lastname: Hirata
authors.forename: Minoru
authors.initials: M
authors.lastname: Ono
authors.forename: Ryozo
authors.initials: R
authors.lastname: Nagai
mesh_terms: D000368:Aged; D000787:Angina Pectoris / Q000209:etiology; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D001327:Autoimmune Diseases / Q000188:drug therapy*; D003323:Coronary Aneurysm / Q000000981:diagnostic imaging / Q000209:etiology; D017023:Coronary Angiography; D003324:Coronary Artery Disease / Q000150:complications / Q000000981:diagnostic imaging / Q000188:drug therapy*; D023921:Coronary Stenosis / Q000000981:diagnostic imaging / Q000209:etiology; D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications / Q000188:drug therapy*; D007074:Immunoglobulin G; D008297:Male; D010195:Pancreatitis / Q000150:complications; D010309:Parotitis / Q000150:complications; D011239:Prednisolone / Q000627:therapeutic use
chemical_list: D000893:Anti-Inflammatory Agents; D007074:Immunoglobulin G; D011239:Prednisolone