Explore chapters and articles related to this topic
Arteropathies, Microcirculation and Vasculitis
Published in Mary N. Sheppard, Practical Cardiovascular Pathology, 2022
Behcet's disease (BD) is a multisystem disease of unknown aetiology characterized by chronic relapsing oro-genital ulcers, uveitis, and systemic involvement including articular, GI, cardiopulmonary, neurological and vascular pathology. It can occur in vessels of any size or type and venous manifestations are frequent. Arterial lesions include aneurysms, occlusions and stenosis. Patients are often male and younger than those with other types of vasculitis as well as individuals of Mediterranean, Middle Eastern or Far Eastern descent. The HLA-B51 gene allele is often associated with Behcet's disease, but not everyone with the gene will develop the disease. Commonly affected organs include the eyes, the mouth and the genitals. There is mixed inflammation of vessels with aneurysm formation and luminal thrombosis. The long-term outcome of arterial lesions in BD is poor, especially in the case of occlusive lesions and associated venous involvement. The use of immunosuppressants improves the prognosis.12
Oral examinations
Published in Deepak Subedi, Marialena Gregoriades, En Hsun Choi, John T Murchison, Graham McKillop, A Complete Guide to the Final FRCR 2B, 2011
Deepak Subedi, Marialena Gregoriades, En Hsun Choi, John T Murchison, Graham McKillop
Aphthoid ulcers are the earliest radiological sign of Crohn’s disease. Infections such as tuberculosis and amoebiasis, and inflammatory conditions such as Behçet’s disease can also produce aphthoid ulceration, but these conditions are rare in the Western European population. However, aphthoid ulcers do not occur in ulcerative colitis. Behçet’s disease is associated with HLA-B51, and clinical manifestations include oral ulcers, skin lesions, genital ulcers, uveitis, recurrent venous thrombosis and a range of neurological involvement. Colonic aphthoid ulcers have been reported in Japanese patients with Behçet’s disease. Gastrointestinal tuberculosis is most commonly manifested in the small bowel and caecum. Tuberculosis can produce ulcers, fistulae and anal fistulae – features that simulate Crohn’s disease. The chronic form of amoebic colitis clinically resembles inflammatory bowel disease. As steroids can potentiate fulminant forms of amoebic colitis, in areas of high incidence it is important to exclude amoebiasis when inflammatory bowel disease is suspected.
Dermatologic Disorders Causing Vulvar Disease
Published in William J. Ledger, Steven S. Witkin, Vulvovaginal Infections, 2017
William J. Ledger, Steven S. Witkin
Aphthous ulcers are a predominant feature of Behçet’s disease, a systematic recurrent immuno-inflammatory vasculitis, mainly affecting young adults. It is characterized by recurrent genital and oral ulcerations, uveitis, and neurological, vascular, pulmonary, and articular manifestations in some individuals.11 The appearance of genital aphthous ulcers is very common. The disease is rare in the Americas and Europe and is most common in Turkey, the Middle East, and the Far East. This geographic distribution strongly suggests a genetic component, and carriage of HLA-B51 has been associated with development of Behçet’s disease.12 The prevailing hypothesis is that when an individual with a genetic susceptibility to develop Behçet’s disease is exposed to a viral or bacterial infection, an autoimmune response is triggered that results in the appearance of clinical symptoms. Interestingly, the concentration of a minor subset of T lymphocytes, gamma delta T cells, is increased in the circulation of individuals with either recurrent aphthous ulcers or Behçet’s disease. Gamma delta T cells recognize, proliferate, and produce pro-inflammatory mediators in response to hsp60, lending further support to the suggestion that hsp60 might be a target antigen for development of autoimmunity leading to aphthous ulcerations. Very recent investigations have also noted the involvement of natural killer cells13 and the pro-inflammatory cytokine, interleukin-33,14 as contributing to Behçet’s disease symptomatology. Since pregnancy favors the predominance of a humoral immune response and inhibition of cell-medicated immunity, it was of interest to determine the effect of pregnancy on Behçet’s disease manifestations. No consistent response was observed. Symptoms improved in some women, became worse in others, and remained the same in a third group of patients.15
HLA-A26 is a risk factor for Behçet’s disease ocular lesions
Published in Modern Rheumatology, 2021
Hiroaki Kato, Masaki Takeuchi, Nobuyuki Horita, Takehito Ishido, Ryuta Mizuki, Tatsukata Kawagoe, Etsuko Shibuya, Kentaro Yuda, Mizuho Ishido, Yuki Mizuki, Takahiko Hayashi, Akira Meguro, Yohei Kirino, Kaoru Minegishi, Hiroto Nakano, Ryusuke Yoshimi, Michiko Kurosawa, Takeshi Fukumoto, Mitsuhiro Takeno, Kazuki Hotta, Takeshi Kaneko, Nobuhisa Mizuki
In 1997, Mizuki et al. scrutinized 31 Greek patients with BD at an ophthalmologic clinic and 30 controls in Greece [8]. This study confirmed that the frequency of HLA-B51 was remarkably high (80.6%) in the patient group as compared to ethnically matched control group (26.7%). In addition, HLA-A26 was identified as a risk allele for BD patients. According to a recently published meta-analysis by Nakamura et al. using the data of 12,752 individuals from independent 15 sources, HLA-A26 positive is a well-known risk of BD with OR of 1.80 (95% CI 1.58–2.06, p < .001) [9]. Nevertheless, HLA-A26 is a weaker risk of BD diagnosis than HLA-B51. The pooled OR of HLA-B51 allele carriers to develop BD compared with non-carriers were 5.78 based on a meta-analysis by de Menthon et al. [5].
Long-term maintenance of the mucosal healing induced by azacitidine therapy in a patient with intestinal Behçet’s-like disease accompanied with myelodysplastic syndrome involving trisomy 8
Published in Immunological Medicine, 2019
Nahoko Tanaka, Hirotake Sakuraba, Hiroto Hiraga, Ko Mayama, Hidezumi Kikuchi, Dai Kishida, Yui Akemoto, Keisuke Hasui, Shinji Ota, Rina Watanabe, Yasuhisa Murai, Takato Maeda, Kosuke Kamata, Tetsuya Tatsuta, Manabu Sawaya, Daisuke Chinda, Tatsuya Mikami, Kazufumi Yamagata, Yoh Ishiguro, Masanori Tanaka, Shinsaku Fukuda
Immunological disorders are observed in 10–15% patients with MDS [2,8]. Especially, MDS patients with intestinal Behçet’s disease-like disease tend to have a high prevalence of trisomy 8. We searched published literature worldwide about intestinal Behçet’s-like disease ascribed to MDS accompanied with trisomy 8 using Pubmed, and we summarized the clinical features of 28 patients’ reports in Table 2. The main subtype of MDS according to the World Health Organization classifications was the low-risk group including 12 patients with refractory anemia and 8 with refractory cytopenia with multilineage dysplasia. Patients’ median age was 54 (range 4–81) years. Twenty of 24 patients (83.3%) had symptoms of Behçet’s disease that preceded MDS. HLA-B51 positivity was found in 5 patients (19.2%). Compared with patients with common Behçet’s disease, those with MDS involving trisomy 8 had more frequent gastrointestinal lesions, less frequent eye lesions, and a lower prevalence of HLA-B51 [2]. However, the association between chromosomal abnormality of trisomy 8 and intestinal Behçet’s-like disease remains unclear. Chen et al. [9] recently reported that upregulated gene expressions of inflammatory cytokines were detected in purified CD34-positive hematopoietic progenitor cells derived from MDS patients with trisomy 8. Therefore, a high prevalence of intestinal Behçet’s-like disease in patients with MDS involving trisomy 8 is closely related with increased inflammatory cytokine signaling pathways.
Concurrent pulmonary artery aneurysms and intracardiac thrombus in a Caucasian man with Behçet’s disease: case report and literature review
Published in Modern Rheumatology Case Reports, 2018
Jean Liew, Daniela Ghetie, Danielle Hosmer, Cailin Sibley
BD is a multisystem inflammatory disorder of unknown aetiology. It is most prevalent in countries along the ancient Silk Road, with an incidence of 62.7 per million in Japan and 8–35 per 10,000 in Turkey; the incidence in the USA is about 1 per 300,000 [40]. HLA-B51 is the main predisposing genetic risk factor. 50–60% of BD patients in Turkey and surrounding countries are positive for HLA-B51, whereas only 15% of those in the US are positive [41]. The diagnosis of BD is supported by the International Study Group (ISG) classification criteria, which consists of recurrent oral ulcers and at least two of the following: recurrent genital ulcers, inflammatory eye disease (anterior or posterior uveitis, or retinal vasculitis), skin manifestations (including erythema nodosum) and a positive pathergy test [40]. The patient described in our report did not meet the ISG criteria for BD, although he did meet the criteria agreed upon by the BD Research Committee of Japan, with three major criteria (recurrent oral ulcers, recurrent genital ulcers and erythema nodosum) and two minor criteria (PAA and epididymitis). Epididymitis and orchitis are the most common urogenital manifestations in males with BD, apart from genital ulcers [42]. Additionally, he was positive for HLA-B51, despite its lower prevalence in Caucasians. Other HLA polymorphisms, such as HLA-A26, were not assessed. The disease manifestations of BD have been thought to be more severe in non-Caucasians; however, insufficient evidence was found to support this contention in a recent systematic review of population-based and comparative studies [43].