バッド・キアリ症候群を伴うベーチェット病とその管理の課題

Behcet's Disease With Budd-Chiari Syndrome and Challenges in Its Management.

• Sudheer K Vuyyuru
• Shivanand Gamanagatti
• Shalimar

抄録

ブッド-キアリー症候群はBehcet病の合併症としては稀であり，下大静脈（IVC）と肝静脈の両方に血栓症を呈することは稀である．我々はBehcet病の若い女性で，急性Budd-Chiari症候群を呈し，下大静脈と肝静脈3本すべてに血栓症を呈した．輸血管ステントを留置し，輸血管ステントを介して経結節性肝内血管系シャントを行った．経口抗凝固薬とステロイドの内服にもかかわらず、経過観察では1年以内に2回の再発血栓症を発症した。彼女の症状はステントの再置換と免疫抑制の増強で改善した。

Budd-Chiari syndrome may rarely occur as a complication of Behcet's disease, and presentation with thrombosis of both inferior vena cava (IVC) and hepatic veins is rarer still. We present a young woman with Behcet's disease who presented with acute Budd-Chiari syndrome, with thrombosis of IVC and all 3 hepatic veins. An IVC stent was placed, followed by a transjugular intrahepatic portosystemic shunt through the IVC stent. On follow-up, despite oral anticoagulants and oral steroids, she developed recurrent thrombosis twice within a 1-year span. Her symptoms resolved with stent revision and increasing immunosuppression.

© 2020 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of The American College of Gastroenterology.