薬物療法を伴わない巨大細胞動脈炎による上行大動脈解離の特異な剖検例

A unique autopsy case of ascending aortic dissection caused by giant cell arteritis without drug therapy.

• Yukari Takase
• Yosuke Murakami
• Jumpei Nishi
• Osamu Tokunaga
• Toshiharu Matsumoto
• Shinichi Aishima

抄録

巨細胞性動脈炎は大・中動脈の肉芽腫性炎症であり，高齢女性に多く発症する．本症例は76歳女性がC反応性蛋白（CRP）高値のため入院して検査を受けたが，胸腹部CTや頭部MRIでは目立った所見は認められなかった．初診から46日目に心タンポナーデにより急死した。病理解剖の結果，死因は上行大動脈に発生した巨細胞動脈炎による急性大動脈解離（Stanford A型）と判明した．組織学的には上行大動脈、胸部下行大動脈、腹部大動脈とその枝に巨大細胞を伴う肉芽腫性血管炎が認められた。興味深いことに，心臓，肝臓，子宮体とその付属物を含む複数の臓器の中小血管にも同様の肉芽腫性血管炎が認められた．我々の知る限りでは，多臓器肉芽腫性変化を伴う巨大細胞性動脈炎はこれまで報告されていない．本稿では，薬物治療を受けていない患者の巨細胞動脈炎を剖検したユニークな症例を報告する．

Giant cell arteritis is a granulomatous inflammation of large and medium-sized arteries, occurring predominantly in older women. In this case, a 76-year-old woman was hospitalized for examination because of a high C-reactive protein (CRP) level, but nothing remarkable was found on thoracicoabdominal computed tomography (CT) or head magnetic resonanse imaging (MRI). On the 46th day from the first visit, she died suddenly due to cardiac tamponade. On pathological autopsy, we found the cause of death to be acute aortic dissection (Stanford type A) due to giant cell arteritis occurred in the ascending aorta. Histologically, granulomatous vasculitis with giant cells was recognized in the ascending aorta, thoracic descending aorta and abdominal aorta and their branches. Interestingly, similar granulomatous vasculitis was also found in the medium and small vessels of other plural organs, including the heart, liver, uterine corpus, and its appendages. To our knowledge, giant cell arteritis with multiple-organ granulomatous changes has not been reported before. We herein reported a unique autopsy case of giant cell arteritis in a patient not treated with medication.

© 2019 Japanese Society of Pathology and John Wiley & Sons Australia, Ltd.