A Case of Localized IgG4-Related Thoracic Periarteritis and Recurrent Nerve Palsy

作者:Takahashi Masao*; Shimizu Takashi; Inajima Tsukasa; Hosoya Yumiko; Takeda Norifumi; Ishizaka Nobukazu; Yamashita Hiroshi; Hirata Yasunobu; Nagai Ryozo
来源:American Journal of the Medical Sciences, 2011, 341(2): 166-169.
DOI:10.1097/MAJ.0b013e3181fbb6f2

摘要

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 F-18-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 F-18-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.

  • 出版日期2011-2