ベーチェット病に合併した急性大動脈弁閉鎖不全症に対する人工弁置換術後3年間経過良好の1例と文献的考察

A Three-years follow-up Case of Operated Aortic Regurgitation Secondary to Behçet's Disease:A case report and review of the literature

　症例は40歳，男性．1991年5月に不全型ベーチェット病（腸管型）による回盲部切除後，サラゾピリン内服中であったが，1992年9月に突然の呼吸困難を主訴に緊急入院．大動脈弁閉鎖不全症（AR）による急性左心不全であった．内科治療抵抗性で大動脈弁置換術を施行したが，左冠尖の破壊と左バルサルバ洞動脈瘤の穿孔があり，組織学的に本症に合致していた．術後10日目に弁置換後のリーク（漏れ）によるARが出現し再手術をした．現在，ステロイドなどの投与により約3年間経過観察中である．ARを伴ったベーチェット病の報告は，われわれの調べた限りでは本例を含めて63例で，34例は人工弁置換を行っていた．術後合併症としてリークが多かった．ARに弁置換術を行って長期経過観察した例は少なく，2年以上の生存が確認されたのは4例であった．ARを合併した本症の長期生存のためには，ステロイドなどによる活動性の抑制が重要と思われた．

A 40-year-old man was admitted with the chief com-plaint of dyspnea on Sep. 11, 1992. He had already been diagnosed as having an incomplete form of Behçet's disease, and salazosulfapyridine therapy has been continued. On auscultation, there were a grade III/VI diastolic high pitch decrescent murmur at the 2nd left sternal border and coarse crackles at the lower lung field. Laboratory data showed an increased white blood cell count of 11,800/mm³ and CRP of 5.3mg/ml. Moder-ate aortic regurgitation (AR) flow wasdetected by color Doppler and cine MRI. Sellers grade was III by DSA (digitized subtraction angiography). Steroid therapy was started. Because of intractable heart failure, aortic valve replacement (St Jude Medical Valve) was perfor-med on Oct 8, 1992. Operative findings showed thedestructed left coronary cusp and a fenestrated aneur-ysm of the left sinus of Valsalva. Pathological findings of aortic valve tissue showed fibrinoid degeneration, monocyte invasion and deposition of hemosiderin. Ten days later, however, AR of Sellers grade II reappeared. The second aortic valve replacement wasperformed on Nov. 2, 1992. After that, he had no symptoms. Labora-tory data showed normalized white bloodcell count and CRP. Steroid therapy was continued. For 3 years, he has had no cardiac symptoms. White blood cell has been 6,300-10,100/mm³. CRP was 0.36-4.53 mg/dl. We have reviewed 62 cases of Behçet's disease with AR in the literature. Most patients were male under the age of 50 years. Thirty-four cases (54%) underwent aortic valve replacement. An important complication was per- valvular leak. Steroid was used for long-term medical therapy in most cases. Only 4 cases survived over 2 years after aortic valve replacement. Careful control of inflammation may be required for long-term survival of Beheet's disease with AR.