The Prevalence and Clinical Characteristics of Esophageal Involvement in Patients with Behçet's Disease: A Single Center Experience in Korea

Yi, Seung Woo; Cheon, Jae Hee; Kim, Joo Hang; Lee, Sang Kil; Kim, Tae Il; Lee, Yong Chan; Kim, Won Ho

doi:10.3346/jkms.2009.24.1.52

Cited by 48 publications

(35 citation statements)

References 16 publications

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“…GI manifestations have been known to cause severe morbidity in patients with BD and are usually referred to as ‘Intestinal Behçet's syndrome’ . The frequency of GI involvement reported in the literature shows a wide range from 0.7% to 50% among different ethnic groups .…”

Section: Discussionmentioning

confidence: 99%

Celiac disease prevalence in a large series of patients with Behçet's disease

et al. 2017

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Section: Discussionmentioning

confidence: 99%

Celiac disease prevalence in a large series of patients with Behçet's disease

et al. 2017

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“…Although intestinal BD is not common, GI manifestation occurs more frequently in East Asia, including Korea, than in the Mediterranean region [ 2 , 36 , 112 , 113 ]. In addition, complications such as intestinal perforation and bleeding considerably increase morbidity and mortality [ 2 , 3 ]; therefore, it is important to manage and treat patients properly.…”

Section: Discussionmentioning

confidence: 99%

Updated treatment strategies for intestinal Behçet’s disease

Park

Cheon

2018

Korean J Intern Med

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Behçet’s disease (BD) is a chronic, idiopathic, relapsing immune-mediated disease involving multiple organs, and is characterized by recurrent oral and genital ulcers, ocular disease, gastrointestinal ulcers, vascular diseases, and skin lesions. In particular, gastrointestinal involvement in BD is followed by severe complications, including massive bleeding, bowel perforation, and fistula, which can lead to significant morbidity and mortality. However, the management of intestinal BD has not yet been properly established. Intestinal BD patients with a severe clinical course experience frequent disease aggravations and often require recurrent corticosteroid and/or immunomodulatory therapies, or even surgery. However, a considerable number of patients with intestinal BD are often refractory to conventional therapies such as corticosteroids and immunomodulators. Recently, there has been a line of evidence suggesting that biologics such as infliximab and adalimumab are effective in treating intestinal BD. Moreover, new biologics targeting proteins other than tumor necrosis factor α are emerging and are under active investigation. Therefore, in this paper, we review the current therapeutic strategies and new clinical data for the treatment of intestinal BD.

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“…Esophageal involvement was found in 4.7% of cases. 30 Endoscopy shows single or multiple punched-out ulcers with well-demarcated edges and relatively flat bottoms. Microscopic features include chronic active inflammation with ulceration.…”

Section: Discussionmentioning

confidence: 99%

Primary Esophageal Extranasal NK/T Cell Lymphoma With Biphasic Morphology

Cao²,

Liu

et al. 2015

Medicine

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We report a case of esophageal extranasal NK/T cell lymphoma with biphasic morphologic features revealed by a deep large piecemeal biopsy.A 40-year-old man present with pharyngalgia, dysphagia, recurrent fever, and 5-kg weight loss for 8 months. Endoscopy demonstrated progressing longitudinal ulcers and mucosal bridges along the esophagus. The first and second biopsies obtained superficial mucosa with scattered bland-looking small lymphocytes. A subsequent large piecemeal snare abscission for biopsy showed atypical lymphoid cells infiltrating into the deep lamina propria and muscularis mucosae, whereas the superficial lamina propria was highly edematous with scant small lymphocytes. Immunohistochemical studies confirmed that both underlying atypical cells and superficial small lymphocytes were neoplastic, sharing an identical immunophenotype: positive for CD2, CD3, CD43, CD8, CD56, TIA-1 and granzyme B. Epstein-Barr virus–encoded small RNAs were found in both cells. The histologic findings were diagnostic of primary esophageal extranasal NK/T cell lymphoma. However, the patient developed bone marrow depression during chemotherapy and died of massive cerebral hemorrhage after the first cycle of chemotherapy.Primary esophageal extranodal NK/T cell lymphoma nasal type is extremely rare. We show the biphasic morphology of this disease, which highlights the importance of deep biopsy for accurate diagnosis.

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The Prevalence and Clinical Characteristics of Esophageal Involvement in Patients with Behçet's Disease: A Single Center Experience in Korea

Cited by 48 publications

References 16 publications

Celiac disease prevalence in a large series of patients with Behçet's disease

Celiac disease prevalence in a large series of patients with Behçet's disease

Updated treatment strategies for intestinal Behçet’s disease

Primary Esophageal Extranasal NK/T Cell Lymphoma With Biphasic Morphology

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