title: A suspected case of coronary periarteritis due to IgG4-related disease as a cause of ischemic heart disease.
abstract: IgG4-related disease (IgG4-RD) is a systemic inflammatory disease characterized by marked infiltration of IgG4-positive (+) plasma cells into affected organs, but the concept of this disease has only recently been established. Coronary vasculitis is a rare disease that can cause sudden death, and it has recently been reported that IgG4-RD may be associated with vasculitis, including periarteritis and coronary disease. In this paper we report an autopsy case of sudden death of a man in his thirties, in which coronary periarteritis with features of IgG4-related periarteritis was detected. IgG4-RD was suspected from the presence of the following histopathological features: (1) markedly thickened adventitia and marked infiltration of the adventitia and periarterial fat by lymphocytes and plasma cells; and (2) infiltration of IgG4-positive plasma cells (ratio of IgG4+ cells to IgG4+ cells of >40 %, 50 IgG4+ plasma cells per high-power field) on immunostaining. The etiology and pathophysiology of IgG4-RD and IgG4-related periarteritis are still unclear, and further investigation of these conditions and their association with coronary lesions is needed. Careful consideration should be given to the possible presence of IgG4-RD when forensic pathologists encounter cases of sudden death accompanied by coronary periarteritis.
authors: Department of Legal Medicine, Graduate School of Medicine, Chiba University, 1-8-1 Inohana, Chuo-ku, Chiba, 260-8670, Japan, goinokuchi@chiba-u.jp.
authors: Go
authors: G
authors: Inokuchi
authors: Mutsumi
authors: M
authors: Hayakawa
authors: Takashi
authors: T
authors: Kishimoto
authors: Yohsuke
authors: Y
authors: Makino
authors: Hirotaro
authors: H
authors: Iwase
mesh_terms: D000328:Adult; D001167:Arteritis / Q000000981:diagnostic imaging / Q000209:etiology* / Q000276:immunology / Q000473:pathology; D001344:Autopsy; D015415:Biomarkers / Q000032:analysis; D002423:Cause of Death; D017023:Coronary Angiography / Q000379:methods; D003324:Coronary Artery Disease / Q000000981:diagnostic imaging / Q000209:etiology* / Q000276:immunology / Q000473:pathology; D003331:Coronary Vessels / Q000000981:diagnostic imaging / Q000276:immunology* / Q000473:pathology; D016757:Death, Sudden, Cardiac / Q000209:etiology* / Q000473:pathology; D017809:Fatal Outcome; D049429:Forensic Pathology / Q000379:methods; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis*; D007249:Inflammation / Q000150:complications* / Q000276:immunology; D008297:Male; D014057:Tomography, X-Ray Computed
chemical_list: D015415:Biomarkers; D007074:Immunoglobulin G