A case of localized IgG4-related thoracic periarteritis and recurrent nerve palsy.
Periarteritis, including periaortitis, is a systemic disorder characterized by an excessive fibroinflammatory reaction that can result in the compromise of great vessels and periarterial/periaortic structures. Recent studies have suggested that IgG4-related inflammation may play a role in chronic periaortitis. These pathologic conditions might represent a systemic disorder with fibrotic reaction rather than local inflammation. In this report, the authors describe a case of a 31-year-old man with marked periaortic fibrous thickening localized to the aortic arch, which was histologically and serologically proven to be IgG4 related. Positron emission tomography showed increased ¹⁸F-fluorodeoxyglucose uptake at this region. Histologic examination revealed infiltration of lymphoplasmacytes and marked fibrosis with numerous IgG4-positive plasma cells. The serum concentration of IgG4 was 263 mg/dL. The size of the periaortic mass and ¹⁸F-fluorodeoxyglucose uptake at this region markedly decreased under corticosteroid therapy. This case suggests that IgG4-related periarteritis can also occur as a solitary focus in the cardiovascular system.
Department of Cardiovascular Medicine, The University of Tokyo, Hongo, Bunkyo-ku, Japan. masaotakahashi-gi@umin.ac.jp
Masao
M
Takahashi
Takashi
T
Shimizu
Tsukasa
T
Inajima
Yumiko
Y
Hosoya
Norifumi
N
Takeda
Nobukazu
N
Ishizaka
Hiroshi
H
Yamashita
Yasunobu
Y
Hirata
Ryozo
R
Nagai
D000328:Adult; D001013:Aorta, Thoracic / Q000000981:diagnostic imaging / Q000276:immunology* / Q000473:pathology*; D001167:Arteritis / Q000000981:diagnostic imaging / Q000276:immunology* / Q000473:pathology*; D019788:Fluorodeoxyglucose F18; D006801:Humans; D007074:Immunoglobulin G / Q000378:metabolism*; D008297:Male; D009422:Nervous System Diseases / Q000209:etiology; D049268:Positron-Emission Tomography; D019275:Radiopharmaceuticals; D012008:Recurrence
D007074:Immunoglobulin G; D019275:Radiopharmaceuticals; D019788:Fluorodeoxyglucose F18