• Gut and Liver
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• 제12권6호
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• pp.623-632
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• 2018

Intestinal Behçet's disease is a rare, immune-mediated chronic intestinal inflammatory disease; therefore, clinical trials to optimize the management and treatment of patients are scarce. Moreover, intestinal Behçet's disease is difficult to treat and often requires surgery because of the failure of conventional medical treatment. Administration of anti-tumor necrosis factor-${\alpha}$, a potential therapeutic strategy, is currently under active clinical investigation, and evidence of its effectiveness for both intestinal Behçet's disease and inflammatory bowel diseases has been accumulating. Here, we review updated data on current experiences and outcomes after the administration of anti-tumor necrosis factor-${\alpha}$ for the treatment of intestinal Behçet's disease. In addition to infliximab and adalimumab, which are the most commonly used agents, we describe agents such as golimumab, etanercept, and certolizumab pegol, which have recently been shown to be effective in refractory intestinal Behçet's disease. This review also discusses safety issues associated with anti-tumor necrosis factor-${\alpha}$, including vulnerability to infections and malignancy.

• Investigative Magnetic Resonance Imaging
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• 제25권4호
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• pp.332-337
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• 2021

Behçet's disease is a chronic inflammatory disorder involving vessels of various sizes and organs, including the skin, joints, gastrointestinal tract, lungs, and cardiovascular system. The etiology of Behçet's disease is unclear, and clinical diagnosis is important in the absence of definitive laboratory or pathological findings diagnostic of Behçet's disease. Cardiac involvement is rare but might present as endocarditis, myocarditis, pericarditis, or intracardiac thrombosis. This report presents a case of Behçet's disease involving the heart in a 22-year-old man with unusual manifestations of right ventricular fibrosis and intracardiac thrombosis. Cardiac magnetic resonance imaging revealed multiple intracardiac thrombi and delayed diffuse subendocardial enhancement involving the right ventricle. No peripheral eosinophilia was detected. Endomyocardial biopsy showed mixed inflammatory cell infiltrates. Based on the patient's clinical history of oral ulcer and arthritis, a diagnosis of Behçet's disease was made considering the clinical, radiological, and histological findings. Intracardiac thrombi and endomyocardial fibrosis are rare manifestations of Behçet's disease, and the diagnosis is often a clinical challenge. Early diagnosis is important for appropriate management. Behçet's disease should be considered in the differential diagnosis of patients with intracardiac thrombosis and endomyocardial fibrosis of the right chamber.

• Journal of Korean Neurosurgical Society
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• 제50권1호
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• pp.68-71
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• 2011

We present a case of Neuro-Behçet's disease with an unpredictable clinical course. A 47-year-old man was admitted to the neurosurgery department of our hospital with a mild headache. Three days after admission, his consciousness suddenly decreased and respiratory distress progressed rapidly. A brain MRI revealed that the previously observed abnormal signal had extended markedly to both the thalamic areas and the entire brain stem, and the surrounding brain parenchyma were compressed by cerebral edema. Based on the patient's symptoms of recurrent oral and genital ulcers, skin lesions, and uveitis, a rheumatologist made a diagnosis of Behçet's disease with CNS involvement. The patient was treated with high-dose methylprednisolone with respiratory assistance in the intensive care unit for 9 days and his neurologic symptoms improved remarkably. Neuro-Behçet's disease must be considered in the differential diagnosis in rapidly deteriorated young neurological patients along with a stroke, low-grade glioma, multiple sclerosis, and occlusive venous disease.

• Tuberculosis and Respiratory Diseases
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• 제52권4호
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• pp.405-410
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• 2002

저자들은 37세 여자환자에서 반복적인 구강궤양 증상 외에 베체트병을 진단하는데 필요한 특별한 주증상이 없이 폐동맥류의 발현으로 용의형 베체트병에 의한 폐혈관합병증의 병발임을 알게된 본 증례를 통해, 드물지만 특별한 병력이 없이 폐동맥류가 발현된 환자에서 베체트병의 폐침범 가능성을 고려하여야 함을 문헌고찰과 함께 보고하는 바이다.

• Tuberculosis and Respiratory Diseases
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• 제56권6호
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• pp.657-663
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• 2004

저자들은 베체트병을 진단받고 치료 받던 환자가 안면부종, 상지부종으로 내원 상대정맥 증후군으로 진단 후 혈전용해제와 스테로이드 치료로 호전을 보인 1예를 경험 하였기에 문헌고찰과 함께 보고하는 바이다.

• Intestinal research
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• 제11권3호
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• pp.155-160
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• 2013

Behçet's disease (BD) is a systemic immunological disorder characterized by recurrent mucosal ulcerative lesions including oral and genital ulcerations in association with skin and ocular involvements. BD also can involve the gastrointestinal tract. Gastrointestinal involvement of BD is one of the major causes of morbidity and mortality for this disease. However, clinical data are quite limited because of the rarity of intestinal BD. Therefore, the management of intestinal BD is heavily dependent on expert opinions and standardized medical treatments of intestinal BD are yet to be established. In this brief review, the authors summarized the currently available medical treatments such as 5-aminosalicylic acids, corticosteroids, immuno-modulators, and anti-TNF agents. Moreover, we sought to suggest a treatment algorithm for intestinal BD based on the recently published and updated data.

• Journal of Korean Neurosurgical Society
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• 제52권5호
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• pp.488-490
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• 2012

Behçet's disease (BD) is an inflammatory systemic disorder with oral and genital ulcers, as well as ophthalmologic and cutaneous symptoms. Neurological manifestations in BD represent between 2.2% to 50% of the cases. The 25-year-old male patient, diagnosed with BD three years earlier, was admitted to our clinic with complaints of recurrent headaches. Tumor-like-parenchimal involvement was detected on a cranial magnetic resonance imaging. The lesion was removed surgically and then he suffered from right hemiparesis and epilepsy. Pathological examination of the lesion noted a demyelinating non-tumoural etiology. A neuro-Beh$\check{g}$et's case with parenchymal involvement has been examined in light of the literature, in terms of a tumor and a demyelinating disease differential diagnosis.

• Tuberculosis and Respiratory Diseases
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• 제43권5호
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• pp.763-773
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• 1996

연구배경: Behçet 증후군은 전신적인 장기에 혈관염에 의한 다양한 병변을 일으키는 원인미상의 질환이다. Behçet 증후군에서 폐침범은 약 5%에서 발생하고, 주로 폐동맥염에 의한 폐동맥류, 혈전증, 폐출혈 등으로 나타나며, 이는 예후에 영향을 미치는 중요한 인자가 된다. 이에 저자 등은 Behçet 증후군 환자에서 폐침범의 유병율, 임상적 소견, 방사선 소견, 치료경과 및 예후 등에 관한 전반적인 고찰을 시행해 보고자 한다. 방법: 1986년부터 1995년까지 본원에 입원한 Behçet 증후군 환자 총 487예중 폐침범의 소견을 보인 10예를 대상으로 하였으며, 폐침범의 진단은 중상 및 이학적 소견을 토대로 하여 단순 흉부방사선, 흉부 전산화단층촬영, 폐환기-관류주사 및 폐동맥조영술 등에 의거하였다. 결과: 1) 전체 Behçet 증후군 환자에서 폐침범의 유병률은 2%(10/487) 였다. 평균연령은 34세, 남녀비는 8 : 2 였다. 객혈은 5 예에서 나타났고, 이중 대량객혈은 2예에서 관찰되었다. 다른 호흡기 증상으로는 기침이 6예, 호홉곤란이 4예, 흉통이 2예에서 나타났다. 구강궤양은 전예, 외음부궤양은 9예, 피부병변은 7예, 안병변은 6예에서 나타났다. 2) 검사실 소견상 특이한 것은 없었고, 단순 흉부방사선 소견상 다발성 경화가 6예, 원형 결절 또는 종괴양 음영이 4예에서 발견되었고 2 예는 정상소견이었다. 흉부 전산화단층촬영은 8예에서 시행하였는데 이중 6예에서 다발성 경화의 소견을 보였고, 4예에서 폐동맥류를 관찰할 수 있었다. 폐관류 또는 환기-관류주사는 8예에서 시행하여 7예에서 다발성 결손 및 불일치를 보였고, 특히 정상 흉부 단순방사선소견을 보인 2 예에서 다발성 환기-관류 불일치의 소견을 보였다. 폐동맥조영술은 3예에서 시행하여, 2예에서 폐동맥류를 관찰할 수 있었다. 3) 치료는 colchicine, 스테로이드, cyclophosphamide, 항응고제 등을 사용하였고, 1예에서 폐동맥류의 절제술을 시행하였다. 4) 10예 중 9예를 추적관찰한 결과, 3예가 호전되어 외래 경과관찰 중이며, 2예는 악화되어 가료중이고, 4예가 대량객혈, 대량 폐색 전증 및 패혈증 등으로 사망하였다. 결론: Behçet 증후군의 폐침범은 주로 혈관염에 의한 출혈, 동맥류의 형성, 및 혈전증 등으로 나타나며, 그 발병율은 낮지만 대량객혈 및 호흡부전증 등을 초래하여, 질환의 경과를 악화시키고 사망을 초래할 수 있는 주원인이 되므로, 이의 조기진단이 필요하며 적극적인 치료로서 예후의 향상을 기대할 수 있을 것이다.

• Gut and Liver
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• 제12권6호
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• pp.674-681
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• 2018

Background/Aims: The cumulative surgery rate and postoperative relapse of intestinal Behçet's disease (BD) have been reported to be high. This study aimed to establish a scoring system based on follow-up endoscopic findings that can predict intestinal BD recurrence after surgery. Methods: Fifty-four patients with intestinal BD who underwent surgery due to bowel complications and underwent follow-up colonoscopy were retrospectively investigated. Their clinical data, including colonoscopic findings, were retrieved. Classification and regression tree analysis was used to develop an appropriate endoscopic classification model that can explain the postsurgical recurrence of intestinal BD most accurately based on the following classification: e0, no lesions; e1, solitary ulcer <20 mm in size; e2, solitary ulcer ${\geq}20mm$ in size; and e3, multiple ulcers regardless of size. Results: Clinical relapse occurred in 37 patients (68.5%). Among 38 patients with colonoscopic recurrence, only 29 patients had clinically relapsed. Multivariate analysis identified higher disease activity index for intestinal BD at colonoscopy (hazard ratio [HR], 1.013; 95% confidence interval [CI], 1.005 to 1.021; p=0.002) and colonoscopic recurrence (HR, 2.829; 95% CI, 1.223 to 6.545; p=0.015) as independent risk factors for clinical relapse of intestinal BD. Endoscopic findings were classified into four groups, and multivariate analysis showed that the endoscopic score was an independent risk factor of clinical relapse (p=0.012). The risk of clinical relapse was higher in the e3 group compared to the e0 group (HR, 6.284; 95% CI, 2.036 to 19.391; p=0.001). Conclusions: This new endoscopic scoring system could predict clinical relapse in patients after surgical resection of intestinal BD.

• Tuberculosis and Respiratory Diseases
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• 제43권1호
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• pp.96-101
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• 1996

저자들은 과거력상 반복되는 구강내 아프타성 궤양 및 결절홍반양 피부병변이 있었고, 객혈을 주소로 내원한 37세 남자환자에서 혈관조영술상 다발성 폐동맥류, 심부 정맥의 협착 및 폐색소견이 관찰되었고, 조직학적으로는 폐혈관염 소견을 보여, Hughes-Stovin 증후군으로 진단하였으며, 코일을 이용한 폐동맥색전술로 폐동맥류를 성공적으로 치료하였기에 문헌고찰과 함께 보고하는 바이다.