title: Immunoglobulin G4-related multiple systemic aneurysms and splenic aneurysm rupture during steroid therapy.
abstract: Immunoglobulin G4 (IgG4)-related disorders in various organs have recently been described, but multiple systemic aneurysms have not yet been reported. Here, we present a 68-year-old Japanese man with multiple systemic aneurysms and tumor-forming pericoronary arteritis who was undergoing low-dose corticosteroid therapy. Elevated serum IgG4 (2390 mg/dL) and IgG4-positive plasmacyte infiltration in the salivary glands led to a diagnosis of IgG4-related disease. High-dose corticosteroid therapy was initiated, whereupon the inflammatory lesions shrank. However, the large, well-developed common hepatic aneurysm and splenic aneurysm did not change. Our patient died of splenic aneurysm rupture in the sixth month of treatment. The autopsy revealed IgG4-positive plasmacyte infiltration in the coronary wall and a thinned splenic aneurysm wall. This case suggests that early high-dose corticosteroid therapy may be necessary for the treatment of IgG4-related cardiovascular disorders. A minor salivary gland biopsy might facilitate the early diagnosis of IgG4-related disease even if (18)F-fluorodeoxyglucose positron emission tomography provides no inflammatory findings.
authors.affiliation: Department of Cardiovascular Medicine, The University of Tokyo, Tokyo 113-8655, Japan.
authors.forename: Miyu
authors.initials: M
authors.lastname: Tajima
authors.forename: Yukio
authors.initials: Y
authors.lastname: Hiroi
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Takazawa
authors.forename: Hironori
authors.initials: H
authors.lastname: Muraoka
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Iwata
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Yamashita
authors.forename: Yasunobu
authors.initials: Y
authors.lastname: Hirata
authors.forename: Ryozo
authors.initials: R
authors.lastname: Nagai
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000368:Aged; D017542:Aneurysm, Ruptured / Q000276:immunology*; D001167:Arteritis / Q000150:complications* / Q000188:drug therapy* / Q000276:immunology; D001327:Autoimmune Diseases / Q000150:complications* / Q000188:drug therapy / Q000276:immunology; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology; D008297:Male
chemical_list: D000305:Adrenal Cortex Hormones; D007074:Immunoglobulin G
keywords: Aneurysm; Immunoglobulin G4; Rupture; Salivary gland biopsy; Steroid