腸管ベーチェット病の腸管病変

回盲部に打ち抜きの巨大円形潰瘍が認められる。潰瘍周囲の炎症はほとんどない。
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クローン病の大腸病変

上行結腸に敷石状所見を伴う縦走潰瘍が認められる。
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腸結核の腸管病変

上行結腸に輪状傾向の潰瘍が認められる。
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ベーチェット病の症状と特定疾患認定基準

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ヒュミラとレミケードの比較

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腸管ベーチェット病の注腸造影写真

大腸部分切除後の吻合部潰瘍。中央に円形のバリウム斑が認められる。
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腸管ベーチェット病・単純性潰瘍患者の手術後または初診時からの臨床的に再燃するまでの期間

手術例では手術後1年で62％、2年で75%の症例で再燃しているのに対し、非手術例では初診後2年で25％の症例にでのみ再燃が認められなかった。
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1: Analysis of clinical course and long-term prognosis of surgical and nonsurgical patients with intestinal Behçet's disease.
著者: M Naganuma, Y Iwao, N Inoue, T Hisamatsu, H Imaeda, H Ishii, T Kanai, M Watanabe, T Hibi
雑誌名: Am J Gastroenterol. 2000 Oct;95(10):2848-51. doi: 10.1111/j.1572-0241.2000.03198.x.
Abstract/Text: OBJECTIVE: Much remains unknown about the pathogenesis of intestinal Behçet's disease. The majority of these patients are treated with surgical intervention, although it has been recently reported that a number of medical treatments are sometimes effective. Only few studies, however, have ever been undertaken to analyze the long-term prognosis of this disease. In this study, we analyzed the clinical course and the recurrences after initial therapy in patients with intestinal Behçet's disease.
METHODS: We investigated 20 patients (surgical group, n = 8; nonsurgical group, n = 12) for whom the clinical courses were known for > or = 2 yr (2-23 yr).
RESULTS: The surgical group tended to have higher rates of complications such as ocular and ileal lesions than the nonsurgical group. In the surgical group, 75% of the patients recurred (and were readmitted) within 2 yr, and 37.5% of the patients required reoperation for intestinal obstruction because of ulcer at the anastomosis. The percentage of peripheral CD8+ DR+ lymphocytes in the recurrent group (10.4% +/- 2.5%) was significantly higher than that in the nonrecurrent group (4.3% +/- 1.2%, p < 0.05).
CONCLUSIONS: Our results indicate that more extensive disease involving the ileum and ocular lesions are markers of severity and progression to surgical crisis, and that patients requiring surgery suffer more frequent recurrences. Furthermore, an increased percentage of peripheral CD8+ DR+ lymphocytes may be a risk factor for disease recurrence.
Am J Gastroenterol. 2000 Oct;95(10):2848-51. doi: 10.1111/j.1572-0241....

インフリキシマブが有効であった症例の腸管内視鏡所見

臨床症状を有する腸管ベーチェット病6例に対してインフリキシマブを投与し、うち4例で有効であること（表[ID0663]）を報告した。インフリキシマブ投与後、劇的に症状が改善し、潰瘍が瘢痕化した
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1: Efficacy of infliximab for induction and maintenance of remission in intestinal Behçet's disease.
著者: Makoto Naganuma, Atsushi Sakuraba, Tadakazu Hisamatsu, Hiroki Ochiai, Hirotoshi Hasegawa, Haruhiko Ogata, Yasushi Iwao, Toshifumi Hibi
雑誌名: Inflamm Bowel Dis. 2008 Sep;14(9):1259-64. doi: 10.1002/ibd.20457.
Abstract/Text: BACKGROUND: Intestinal Behçet disease (BD) is characterized by intestinal inflammation with round and oval ulcers associated with gastrointestinal symptoms. Although several cases have been reported that infliximab is effective for induction of remission, the efficacy of infliximab for maintaining remission is unknown.
METHODS: Six cases with fulminant intestinal BD were treated with infliximab. All patients were steroid-dependent and refractory to immunosuppressants; 3 patients were treated with 6-mercaptopurine, 1 patient with azathioprine, 1 patient with cyclosporine A, and 1 patient with methotrexate.
RESULTS: Four patients achieved remission by infliximab and all of these patients maintained remission with scheduled treatments of infliximab, with the longest duration of remission being about 3 years. Another 2 patients with ileal ulceration required surgery; however, 1 patient has maintained remission by scheduled treatment of infliximab for 2 years after surgery.
CONCLUSIONS: Infliximab appears to offer an option for fulminant intestinal BD to induce and maintain remission, although a randomized control trial is needed.
Inflamm Bowel Dis. 2008 Sep;14(9):1259-64. doi: 10.1002/ibd.20457.

ステロイド・イムラン無効例へのインフリキシマブを投与の結果

臨床症状を有する腸管ベーチェット病6例に対してインフリキシマブを投与し、うち4例で有効であった
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1: Efficacy of infliximab for induction and maintenance of remission in intestinal Behçet's disease.
著者: Makoto Naganuma, Atsushi Sakuraba, Tadakazu Hisamatsu, Hiroki Ochiai, Hirotoshi Hasegawa, Haruhiko Ogata, Yasushi Iwao, Toshifumi Hibi
雑誌名: Inflamm Bowel Dis. 2008 Sep;14(9):1259-64. doi: 10.1002/ibd.20457.
Abstract/Text: BACKGROUND: Intestinal Behçet disease (BD) is characterized by intestinal inflammation with round and oval ulcers associated with gastrointestinal symptoms. Although several cases have been reported that infliximab is effective for induction of remission, the efficacy of infliximab for maintaining remission is unknown.
METHODS: Six cases with fulminant intestinal BD were treated with infliximab. All patients were steroid-dependent and refractory to immunosuppressants; 3 patients were treated with 6-mercaptopurine, 1 patient with azathioprine, 1 patient with cyclosporine A, and 1 patient with methotrexate.
RESULTS: Four patients achieved remission by infliximab and all of these patients maintained remission with scheduled treatments of infliximab, with the longest duration of remission being about 3 years. Another 2 patients with ileal ulceration required surgery; however, 1 patient has maintained remission by scheduled treatment of infliximab for 2 years after surgery.
CONCLUSIONS: Infliximab appears to offer an option for fulminant intestinal BD to induce and maintain remission, although a randomized control trial is needed.
Inflamm Bowel Dis. 2008 Sep;14(9):1259-64. doi: 10.1002/ibd.20457.

チオプリン製剤を使用している患者における臨床的再燃率

韓国のグループはチオプリン製剤を使用している患者における臨床的再燃率を調査し、2年で29％、5年で51％であることを報告した
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1: Clinical outcomes and prognostic factors for thiopurine maintenance therapy in patients with intestinal Behcet's disease.
著者: Yoon Suk Jung, Jae Hee Cheon, Sung Pil Hong, Tae Il Kim, Won Ho Kim
雑誌名: Inflamm Bowel Dis. 2012 Apr;18(4):750-7. doi: 10.1002/ibd.21757. Epub 2011 May 25.
Abstract/Text: BACKGROUND: To date, there have been no studies focusing on the efficacy of thiopurine therapy in intestinal Behcet's disease (BD). We conducted this study to investigate clinical outcomes and predictors of clinical relapse in intestinal BD patients receiving thiopurine maintenance therapy.
METHODS: We reviewed the medical records of all patients with intestinal BD who received thiopurine therapy in a single tertiary academic medical center between March 1986 and October 2010. The cumulative probabilities of clinical relapse after remission were calculated using the Kaplan-Meier method. Predictors of clinical relapse were identified by univariate analysis using the log-rank test and by multivariate analysis using Cox proportional hazards regression models.
RESULTS: Of a total of 272 patients with intestinal BD, 67 (24.6%) received their first course of thiopurine therapy at our center. Thirty-nine (58.2%) of the 67 patients constantly received thiopurines for maintaining medically or surgically induced remission. The cumulative relapse rates at 1 year, 2 years, 3 years, and 5 years after remission were 5.8%, 28.7%, 43.7%, and 51.7%, respectively. On multivariate analysis, a younger age (<25 years) at diagnosis and a lower hemoglobin level (<11 g/dL) were independent predictive factors for relapse in intestinal BD patients receiving thiopurine maintenance therapy.
CONCLUSIONS: Thiopurine therapy showed a relatively good effect for maintenance of remission in intestinal BD patients. However, a younger age at diagnosis and a lower hemoglobin level were associated with a poor response to thiopurines, necessitating early adoption of effective alternative therapeutic options in these risk groups.

Copyright © 2011 Crohn's & Colitis Foundation of America, Inc.
Inflamm Bowel Dis. 2012 Apr;18(4):750-7. doi: 10.1002/ibd.21757. Epub ...

チオプリン製剤による外科手術後腸管ベーチェット病の長期予後への影響

外科手術後にアザチオプリンを服用した群としなかった群で再燃率を比較したところアザチオプリン使用群では5年でわずか24%しか再燃しなかったのに対し、非使用群では約半数の症例で再燃した
 
参考文献：Choi IJ, Kim JS, Cha SD, Jung HC, Park JG, Song IS, Kim CY. Long-term clinical course and prognostic factors in intestinal Behçet's disease.Dis Colon Rectum. 2000 May;43(5):692-700.PMID: 10826433
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腸管ベーチェット病治療のアルゴリズム

  1. 5-ASA:5-アミノサリチル酸
  1. AZA：アザチオプリン
  1. 6-MP：6-メルカプトプリン

腸管ベーチェット病診断のアルゴリズム

  1. NSAID:非ステロイド抗炎症薬