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免疫グロブリンG4(IgG4)関連疾患は全身臓器に同時性あるいは異時性に腫大や肥厚,結節性変化をきたすことで知られている.近年,動脈周囲や腎盂尿管周囲にも同様の病変をきたすことが明らかになり,IgG4関連動脈周囲炎および後腹膜線維症の概念が提唱されているが,包括的診断基準による確定診断に侵襲度の高い組織採取が必要とされるため,容易に診断することはむずかしいとされている1).われわれは,縦隔腫瘍との鑑別を要し,胸部大動脈周囲に発生したIgG4関連大動脈周囲炎と準確定診断された症例を経験したので,報告する.
A 42-year-old man with a history of suspected of Behcet’s disease underwent oral steroid treatment. During follow-up, chest X-ray revealed an abnormal shadow of the mediastinum. Chest computed tomography (CT) showed a circumferential tumor around the descending thoracic aorta. Enhanced CT showed a lowly and uniformly enhanced tumor at delay phase. A mediastinal tumor was suspected, which prompted a biopsy of the periaortic tumor by video-assisted thoracic surgery (VATS). Histopathological diagnosis showed numerous immunogloblin G4 (IgG4)-positive plasma cells suggesting the possibility of IgG4-related periaortitis. However, based on the diagnostic criteria, the case was comprehensively diagnosed as probable IgG4-related periaortitis, steroid treatment may have affected blood IgG4-positive cells and tissues.
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