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류마티스내과/혈관염

베체트 식도궤양, Esophageal ulcer in esophagus

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30대

# 베체트, 식도 궤양. 과거력

편도염이 오는 것처럼 목의 이물감이 있다

36.9

스테로이드. 4-5년 전 복용한 적 있음 (베체트 식도궤양 진단 당시)

2020.11.25 음식을 먹으면 목이 막힌 것 같고 최근 속이 쓰렸다. 가래가 올라온다. 최근에 과식을 하고 체한 것 같았다. 현재 복용 중인약 없다.

2021.01.15 식도가 쓰려고 잠을 자기가 어렵다. 밤에 자려고 누으면 쓰리고 넘어 오려고 한다.

역류식도염 증상도 있지만 이전 베체트 식도 궤양 당시의 증상과 비슷하다고 하여

이전에 복용하였던 Prednisolone 10 mg, Cochicine 0.6 mg, Rabeprazole 20 mg을 처방하였습니다.

증상이 호전되어 내원하였지만 PPI 때문인지 스테로이드 때문인지...

Glucocorticoids plus azathioprine – Typical starting doses for prednisone for gastrointestinal Behçet syndrome are 0.5 to 1 mg/kg daily. The initial dose of glucocorticoids is usually maintained for at least one month or until symptoms improve before the institution of a taper, which is designed to decrease the daily dose to 10 mg/day within two to three months. Subsequent tapering to discontinuation over an additional two months may proceed if disease control is maintained.

Azathioprine should be instituted at essentially the same time as prednisone. Treatment is begun at 50 mg/day, after testing for mutations in the gene for thiopurine methyltransferase, and increased at weekly intervals until the target dose of 2.5 mg/kg daily is achieved (usually within one month). Azathioprine should be maintained for at least six months, with periodic reassessments of its need and serial endoscopies conducted by the gastroenterologist as appropriate. In an observational study including 37 patients with moderate to severe gastrointestinal manifestations of Behçet syndrome who were prescribed azathioprine initially, remission was observed in 65 percent of patients during a mean follow up of 69 months.

TNF-alpha inhibitors – We generally use TNF-alpha inhibitors in patients for whom treatment with glucocorticoids plus azathioprine has failed to control the disease. TNF-alpha inhibitors are generally used in combination with other therapies, preferably azathioprine. Several reports indicate successful treatment of intestinal Behçet syndrome with infliximab and adalimumab using regimens approximating those approved for the treatment of inflammatory bowel disease. The typical schedule of infliximab administration is 5 mg/kg at zero, two, and six weeks, followed by 5 mg/kg every eight weeks. In a retrospective study of 28 patients with moderate to severe intestinal Behçet syndrome, 54 percent of patients who had received infliximab demonstrated lasting control of disease activity and symptom improvement over the follow-up period (median duration of 30 months). Factors predictive of sustained response included older age at diagnosis, female sex, longer disease duration, concomitant immunomodulator use, and achievement of remission at four weeks.

Sulfasalazine – Though not well studied, sulfasalazine and other aminosalicylate agents are often used to treat gastrointestinal disease in the same fashion as they are used in the treatment of inflammatory bowel disease. Treatment with oral 5-aminosalicyclic acid was studied in 41 patients with intestinal manifestations of Behçet syndrome. At eight weeks in the 28 patients who remained in the study, clinical response occurred in 61 percent and remission in 57 percent. Endoscopic evaluation of 17 patients at 52 weeks demonstrated endoscopic response in 71 percent and remission in 35 percent. In all 41 patients, rescue therapy-free survival occurred in 73 percent and surgery-free survival in 100 percent. As described above, we generally use sulfasalazine in combination with TNF-alpha inhibitors. Pretreatment testing prior to the use of sulfasalazine, including screening patients at high risk for glucose-6-phosphate dehydrogenase (G6PD) deficiency, is discussed in detail separately.

Other – Alternative options include mycophenolate and methotrexate. Total parenteral nutrition, enteral nutrition, and surgery may be used when clinically indicated.

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