胸部下行大動脈に動脈瘤を合併したWegener 肉芽腫症の1 例

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タイトル別名
  • A Case Aneurysm on the Descending Thoracic Aorta in Wegener's Granulomatosis
  • キョウブ カコウ ダイドウミャク ニ ドウミャクリュウ オ ガッペイシタ Wegener ニクガシュ ショウ ノ 1レイ

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type:症例報告

type:Case Report

79 歳男性.発熱,咳嗽で発症し,鼻閉の既往,空洞を伴う両下肺の浸潤影,顕微鏡的血尿,急速進行する腎機能障害およびPR3-ANCA 高値を示し,Wegener 肉芽腫症( WG) と診断された.一時シクロフォスファミド併用を含む,高用量副腎ステロイド療法が開始され,臨床および検査所見は改善した.ステロイド薬は漸減され,3 ヶ月後にはC-ANCA は陰性化した.WG は寛解していたが,8 ヶ月後に胸部下行大動脈瘤形成を認め,その1 ヶ月後に突然大喀血し,死亡した.動脈瘤形成は疾患活動性とは関連なく,経過中注意深い観察の継続を要する.

A 79-year-old man was admitted because of fever andcough. He had been diagnosed with Wegener's granulomatosis(WG) on the basis of 10-year history of stuffy nose,pulmonary infiltrates with cavity formation, rapidly progressedrenal dysfunction with microscopic hematuria, andhigh level of PR3-ANCA. He was treated with high dose ofglucocorticoid( GC) temporarily in combination with cyclophosphamide,accordingly, the clinical manifestation and thelaboratory results had been recovering. The dose of GC wastapered down, and 3 months after the initiation of therapy,C-ANCA testing was negative. Despite remission was obtained,8 months after, chest computed tomography (CT)scan showed aneurysm formation on the descending aorta.One month after that, the patient had an abrupt onset ofcoughing up a massive amount of blood, and died. Aneurysmformation may develop independently of disease activityin WG and may be unpredictable, so that a carefulobservation whether aneurysm is formed should be continuouslyconsidered in the follow-up during the entire course.

identifier:18

identifier:KJ00007071079

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