The oral mucosal and salivary microbial community of Behçet's syndrome and recurrent aphthous stomatitis.

This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License, permitting all non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.Behçet's syndrome (BS) is a multisystem immune-related disease of unknown etiology. Recurrent aphthous stomatitis (RAS) is characterized by the presence of idiopathic oral ulceration without extraoral manifestation. The interplay between the oral microbial communities and the immune response could play an important role in the etiology and pathogenesis of both BS and RAS

A Case of Intestinal Behcet's Disease Similar to Crohn's Colitis

Behcet's disease is a multi-systemic vasculitis and characterized by systemic organ involvement. Although the gastrointestinal and systemic features of Behcet's disease and inflammatory bowel disease overlap to a considerable extent, they are generally viewed as two distinct diseases. A 39-yr-old female was diagnosed as having Behcet's disease. She was admitted to our hospital because of oral and genital ulcer, lower abdominal pain, and frequent diarrhea. Colonosopy showed diffuse involvement of multiple longitudinal ulcers with inflammatory pseudopolyps with a cobblestone appearance and ano-rectal fistula was suspected. These findings are extremely rare in Behcet's disease. However, there were no granulomas, the hallmark of Crohn's colitis. Microscopically, perivasculitis and multiple lymph follicles compatible with Behcet's disease were seen. Although being rarely encountered, multiple longitudinal ulcers, cobblestone appearance, and ano-rectal fistula can develop in Behcet's disease, as in Crohn's colitis. Therefore, Behcet's disease and Crohn's disease may be closely related and part of a spectrum of disease

Remission of intestinal Behçet's disease treated with anti-tumor necrosis factor α monoclonal antibody (Infliximab)

Behçet's disease (BD) is a chronic relapsing multisystem disease characterized by oral ulceration, genital ulceration and ocular lesions. Gastrointestinal involvement is rare, often difficult to treat and associated with a high mortality rate. We treated a 47-year-old Korean man with BD who had a recurrent intestinal ulcer with tumor necrosis factor α antibody (infliximab); he initially underwent right hemicolectomy due to uncontrolled intestinal bleeding. For patients with intestinal BD who fail to respond to conventional treatment, infliximab may be a safe and effective new therapeutic option

Rapidly Progressive Cardiac Manifestation of Behçet's Disease Involving Conduction System and Aortic Valve

Cardiac conduction system impairment is a rare clinical manifestation of Behçet's disease. We report a patient who showed 1st degree atrioventricular block at first presentation, and showed aggravated finding of 3rd degree atrioventricular block on five months later. His cardiac manifestation finally developed to acute severe aortic regurgitation on six months later from his first cardiac manifestation. We observed this rapid progression during 6 months and successfully improved symptom and disease severity of the patient with treatment targeting Behçet's disease

Association of TNFA Promoter Region Haplotype in Behçet's Disease

Although the etiology of Behçet's Disease (BD; MIM 109650) remains to be clearly elucidated, levels of tumor necrosis factor alpha (TNF-α) have been reported to be significantly elevated in BD patients, and TNF-α blockers have been demonstrated to exhibit some degree of therapeutic efficacy for a certain subset of BD sufferers. In this study, we have conducted an analysis of the TNFA haplotypes in the promoter response element that affect the binding affinity of specific transcription factors, in order to characterize their association with the clinical features of BD. Six polymorphisms in the promoter region of TNFA were genotyped in 254 BD patients and 344 control subjects, via the PCR-RFLP technique. TNFA -1031*C, -863*A and -308*G alleles were associated with an increased risk of BD (p=0.030, OR=1.4; p=0.008, OR=1.5; p=0.010, OR=1.8, respectively). The sole TNFA haplotype -1031C-863A-857C-376G-308G-238G, was associated with a 1.6 fold increase in the risk of BD, whereas the TNFA haplotype -1031T-863C-857C-376G-308A-238G was associated with a 0.6 decreased risk of BD. The TNFA -1031*C, -863*A, -857*C and -308*G alleles were significantly associated with BD. The findings of this study, collectively, indicate that TNFA haplotypes in the promoter response elements may exert significant influence on susceptibility to BD