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A case of localized IgG4-related thoracic periarteritis and recurrent nerve palsy
Masao Takahashi1, Takashi Shimizu, Tsukasa Inajima
1Department of Cardiovascular Medicine, The University of Tokyo, Hongo, Bunkyo-ku, Japan. masaotakahashi-gi@umin.ac.jp
Abstract:
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.
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