Spontaneous aortic pseudoaneurysm rupture into the sigmoid colon in Behcet's disease patient

Beh?et's disease(BD) is a multisystem autoimmune disorder characterized by recurrent orogenital ulcers,uveitis,and skin lesions. The vascular manifestations include thrombophlebitis,stenosis,occlusion,and pseudoaneurysm. BD infrequently precipitates aortic pseudoaneurysm rupture into the sigmoid mesocolon and lumen of the adjacent colon. Here we report an extremely rare case of spontaneous abdominal aortic pseudoaneurysm rupture via the sigmoid mesocolon into the lumen of the sigmoid colon in a 37-year-old patient with BD.

Characteristic changes of intestinal flora and its correlation with clinical indexes in patients with Behcet's disease based on TCM syndromes

Objective: To analyze the characteristics of intestinal microflora in patients with Behcet's disease of damp heat syndrome by 16S rRNA technique and its correlation with clinical indexes;Methods: 49 patients with Behcet's disease were divided into Shire group (SR, n =19) and non-shire group (Non-SR, n = 30), and 43 healthy subjects were selected as control group. The basic information and laboratory test indexes of three groups were collected, and fecal samples were collected to detect the diversity and abundance of intestinal flora in each group by 16S rRNA. Results: Compared with Non-SR group, the incidence of ocular involvement was higher in SR group (P<0.05). At the same time, WBC, CRP, ESR and IgG in SR group were significantly higher than those in Non-SR group (P<0.05). The results of alpha diversity showed that the ace, Chao 1 and Shannon indexes of the SR group were significantly lower than those of the healthy group and the Non-SR group, while the Simpson index was significantly higher (P<0.05). Compared with the Non-SR group, the abundance of Actinobacteria phylum increased in the SR group (P<0.05);the level abundance of Collinsella, Intestinimonas and Enterobacteriaceae_ unclassified genus were increased, and the level abundance of Clostridiales_unclassified, Ruminococcaceae_UCG009, and Ruminococcus_gauvre auii_group genus were decreased (P<0.05). Pearson correlation analysis showed that Oscillospira abundance was positively correlated with WBC, CRP, ESR and IgG levels, while Eubacterium and Cetobacterium abundance were negatively correlated with WBC, CRP, ESR and IgG levels. Conclusion: The change of intestinal flora is involved in the pathogenesis of BD. At the same time, there are differences in intestinal flora in different types of BD patients. In addition, the specific flora in intestinal tract is closely related to the serum inflammation and immune indexes of BD.

Network pharmacology approach on the mechanism of Sanwu Huangqin Decoction in treating Behcet's disease

Objective:To analyze the main compounds of Sanwu Huangqin decoction based on the method of network pharmacology and to predict its possible target and mechanism of action in the treatment of Behcet's disease(BD).Methods:TCMSP,TCMID and literatures were used to search the main compounds and their corresponding targets of Sanwu Huangqin Decoction;the disease targets of BD were searched by GeneCards database;the intersection targets of Sanwu Huangqin Decoction and BD were visualized by String database and Cytoscape software;the intersection targets were enriched by GO and KEGG by DAVID database;BD mouse model was established,RT-PCR was used to verify the regulatory effect of Sanwu Huangqin Decoction on the core target genes.Results:A total of 81 main active compounds and 50 drug-disease-target were retrieved from the databases.There were 531 biological function information and 96 signal pathways were enriched by GO and KEGG.Among them,the top 5 signal pathways were Fluid shear stress and atherosclerosis、AGE-RAGE signaling pathway in diabetic complications、IL-17 signaling pathway、TNF signaling pathway、Malaria、Chagas disease(American trypanosomiasis).RT-PCR results showed that the expression of IL-4,IL-1β,IL-6,INF-γmRNA in the spleen of BD mice was significantly up-regulated,and the expression of Caspase 3 and Caspase 8 mRNA was significantly down regulated when compared with the control group(P<0.01).Compared with the model group,the expression of IL-4,IL-1β,IL-6,INF-γmRNA in the Sanwu Huangqin decoction group was significantly down regulated,and the expression of caspase 3 and caspase 8 mRNA was significantly up regulated(P<0.01).Conclusion:Sanwu Huangqin decoction can treat BD through multi-target and multi-channel therapy,and our study and our research provides bioinformatics basis for further revealing its mechanism and treatment.

Effect of Sanwu Huangqin Decoction on IFN-γ and IL-17A levels in mice with Behcet's disease

Objective:To investigate the effect of Sanwu Huangqin Decoction on IFN-γand IL-17A levels in mice with Behcet's disease;Methods:Clinical samples of patients with Behcet's disease were collected and mononuclear cells were isolated.High throughput RNA-seq was used to identify the different genes between Behcet's disease and healthy subjects.Mouse model was constructed by shrimp myosin sensitization and treated with Sanwu Huangqin decoction.The levels of IFN-γand IL-17A in peripheral blood and mononuclear cells were detected by ELISA and flow cytometry.The expression level of TBX21 gene in spleen was detected by RT-PCR;Results:Compared with healthy subjects,31 differential genes were identified in BD patients,of which 19 genes were up-regulated and 12 genes were down regulated.PPI analysis showed that the top 5 genes were TBX21,STAT3,IL15,PRF1 and IFNG.The results of animal experiments showed that the levels of IFN-γand IL-17A in the peripheral blood and PBMC of the model group were significantly higher compared with the control group(P<0.01).Sanwu Huangqin decoction could significantly reduce the levels of IFN-γand IL-17A in the peripheral blood and PBMC of mice(P<0.05 or P<0.01).RT-PCR results showed that the expression of TBX21 mRNA in spleen tissue of model group was significantly higher than that of the control group(P<0.01).Sanwu Huangqin decoction could significantly down-regulate the expression of TBX21 mRNA in spleen(P<0.05 or P<0.01);Conclusion:Sanwu Huangqin decoction reduce the levels of IFN-γand IL-17A by inhibiting the expression of TBX21 mRNA,so as to achieve the effect of treating BD.

Endoscopic findings of gastrointestinal involvement in Chinese patients with Behcet's disease

AIM:To report the incidence,clinical features and outcomes of gastrointestinal(GI)involvement in Behcet’s disease(BD).METHODS:A total of 168 consecutive patients with BD were screened and upper and lower GI endoscopies were performed in 148 patients.Four hundred age-and sex-matched controls were enrolled for comparison.RESULTS:Fifty-two(35.1%)patients had GI lesions.After a mean follow-up of 10 mo,ileocecal ulcers had been confirmed in 20 patients,including active ulcer(s)in 18 patients,but no ileocecal ulceration was found in controls.GI symptoms were present in 14 patients with active ulcer(s),while 4 patients with smaller ulcer were asymptomatic.Endoscopic features of ileocecalulcer were:a single ulcer(50%),larger than 1 cm in diameter(72.2%),and round/oval or volcano-type in shape(83.3%).Compared with patients without GI involvement,less ocular lesions,lower levels of albumin,erythrocyte count and hemoglobin,and higher levels of C-reactive protein and erythrocyte sedimentation rate were confirmed in the intestinal BD group.Four patients had esophageal ulcers in the BD group but no case in controls.The other endoscopic findings were similar between the two groups.The prevalence of Helicobacter pylori infection was similar in both groups.Most patients received an immunomodulator and responded well.CONCLUSION:GI lesions commonly occur in Chinese BD patients.The most frequently involved area is the ileocecal region.Esophageal ulcer might be a rare but unique lesion.

Gastrointestinal Behcet's disease:A review

Beh?et's disease(BD) is an idiopathic, chronic, relapsing, multi-systemic vasculitis characterized by recurrent oral and genital aphthous ulcers, ocular disease and skin lesions. Prevalence of BD is highest in countries along the ancient silk road from the Mediterranean basin to East Asia. By comparison, the prevalence in North American and Northern European countries is low. Gastrointestinal manifestations of Beh?et's disease are of particular importance as they are associated with significant morbidity and mortality. Although ileocecal involvement is most commonly described, BD may involve any segment of the intestinal tract as well as the various organs within the gastrointestinal system. Diagnosis is based on clinical criteria- there are no pathognomonic laboratory tests. Methods for monitoring disease activity on therapy are available but imperfect. Evidence-based treatment strategies are lacking. Different classes of medications have been successfully used for the treatment of intestinal BD which include 5-aminosalicylic acid, corticosteroids, immunomodulators, and anti-tumor necrosis factor alpha monoclonal antibody therapy. Like inflammatory bowel disease, surgery is reserved for those who are resistant to medical therapy. A subset of patients have a poor disease course. Accurate methods to detect these patients and the optimal strategy for their treatment are not known at this time.

Confusing untypical intestinal Behcet's disease:Skip ulcers with severe lower gastrointestinal hemorrhage

Behcet's disease(BD) is a rare and life-long disorder characterized by inflammation of blood vessels throughout the body. BD was originally described in 1937 as a syndrome involving oral and genital ulceration in addition to ocular inflammation. Intestinal BD refers to colonic ulcerative lesions documented by objective measures in patients with BD. Many studies have shown that over 40% of BD patients have gastrointestinal complaints. Symptoms include abdominal pain, diarrhea, nausea, anorexia and abdominal distension. Although gastrointestinal symptoms are common, the demonstration of gastrointestinal ulcers is rare. This so-called intestinal BD accounts for approximately 1% of cases. There is no specific test for BD, and the diagnosis is based on clinical criteria. The manifestations of intestinal BD are similar to those of other colitis conditions such as Crohn's disease or intestinal tuberculosis, thus, it is challenging for gastroenterologists to accurately diagnose intestinal BD in patients with ileocolonic ulcers. However, giant ulcers distributed in the esophagus and ileocecal junction with gastrointestinal hemorrhage are rare in intestinal BD. Here, we present a case of untypical intestinal BD. The patient had recurrent aphthous ulceration of the oral mucosa, and esophageal and ileo-colonic ulceration, but no typical extra-intestinal symptoms. During examination, the patient had massive acute lower gastrointestinal bleeding. The patient underwent ileostomy after an emergency right hemicolectomy and partial ileectomy, and was subsequently diagnosed with incomplete-type intestinal BD by pathology. The literature on the evaluation and management of this condition is reviewed.

A single nucleotide polymorphism in the IL1RL1 gene is associated with Behcet's disease in a Chinese Han population

AIM:To explore the association of single nucleotide polymorphisms(SNPs)in the IL33/IL1RL1 gene region with the susceptibility to Behcet’s disease(BD)in a Chinese Han population.METHODS:A total of eight SNPs in the candidate gene region(rs11792633,rs7025417,rs10975519 and rs1048274 in IL33;rs2310220,rs12712142,rs13424006 and rs3821204 in IL1RL1)were genotyped in783 BD patients and 701 healthy controls by the Sequenom Mass Array i PLEX platform.RESULTS:A statistically significant association was observed between IL1RL1 rs12712142 and BD patients.The frequency of IL1RL1 rs12712142 variant allele A was significantly lower in BD patients than that in controls(OR=0.8,95%CI:0.69-0.94,Pc=0.039);the genotype distribution(Pc=0.043)and additive and dominant genetic model analyses(OR=0.8,95%CI:0.69-0.94,Pc=0.040 and OR=0.72,95%CI:0.58-0.88,Pc=0.011)also indicated a strong association between rs12712142 and BD patients.CONCLUSION:This is the first study to reveal the association between IL1RL1 rs12712142 variant allele A and the decreased risk of BD in the Chinese Han population,indicating a protective role of IL1RL1 in the pathogenesis of BD.

Correlations between endoscopic and clinical disease activity indices in intestinal Behcet's disease

AIM:To develop a novel endoscopic severity model of intestinal Behcet's disease(BD) and to evaluate its feasibility by comparing it with the actual disease activity index for intestinal Behcet's disease(DAIBD).METHODS:We reviewed the medical records of 167 intestinal BD patients between March 1986 and April 2011.We also investigated the endoscopic parameters including ulcer locations,distribution,number,depth,shape,size and margin to identify independent factors associated with DAIBD.An endoscopic severity model was developed using significant colonoscopic variables identified by multivariate regression analysis and its correlation with the DAIBD was evaluated.To determine factors related to the discrepancy between endoscopic severity and clinical activity,clinical characteristics and laboratory markers of the patients were analyzed.RESULTS:A multivariate regression analysis revealed that the number of intestinal ulcers(≥ 2,P = 0.031) and volcanoshaped ulcers(P = 0.001) were predictive factors for the DAIBD.An endoscopic severity model(Y) was developed based on selected endoscopic variables as follows:Y = 47.44 + 9.04 × non-Ileocecal area + 11.85 ×≥ 2 of intestinal ulcers + 5.03 × shallow ulcers + 12.76 × deep ulcers + 4.47 × geographicshaped ulcers + 26.93 × volcano-shaped ulcers + 8.65 ×≥ 20 mm of intestinal ulcers.However,endoscopic parameters used in the multivariate analysis explained only 18.9% of the DAIBD variance.Patients with severe DAIBD scores but with moderately predicted disease activity by the endoscopic severity model had more symptoms of irritable bowel syndrome(21.4% vs 4.9%,P = 0.026) and a lower rate of corticosteroid use(50.0% vs 75.6%,P = 0.016) than those with severe DAIBD scores and accurately predicted disease by the model.CONCLUSION:Our study showed that the number of intestinal ulcers and volcano-shaped ulcers were predictive factors for severe DAIBD scores.However,the correlation between endoscopic severity and DAIBD(r = 0.434) was weak.

Adamantiades-Behcet's disease-complicated gastroenteropathy

Adamantiades-Behcet's disease (ABD) is a chronic,relapsing,systemic vasculitis of unknown etiology.It is more prevalent in populations along the ancient Silk Road from Eastern Asia to the Mediterranean Basin,and most frequently affects young adults between the second and fourth decades of life.ABD-complicated gastroenteropathy is a significant cause of morbidity and mortality,with abdominal pain as the most common symptom.The ileocecal region is affected predominantly,with ulcerations that may lead to penetration and/or perforation,whereas other parts of the gastrointestinal system including the esophagus and stomach can also be affected.Endoscopy is useful to locate the site and extent of the lesions,and tissue biopsy is often warranted to examine the histopathology that is often suggestive of underlying vasculitis of small veins/venules or,alternatively in some cases,nonspecific inflammation.Bowel wall thickening is the most common finding on computed tomography scan.Treatment is largely empirical since well-controlled studies are difficult to conduct due to the heterogeneity of the disease,and the unpredictable course with exacerbation and remission.Corticosteroids with or without other immunosuppressive drugs,such as cyclophosphamide,azathioprine,sulfasalazine,tumor necrosis factor α antagonist or thalidomide should be applied before surgery,except in emergency.

Clinical Characters of Gastrointestinal Lesions in Intestinal Behcet's Disease

Objective To investigate the clinical features,diagnosis methods,therapeutic principles of intestinal Behcet's disease.Methods The clinical data of 45 patients with confirmed intestinal Behcet's disease admitted to Peking Union Medical College Hospital from August 1998 to April 2010 were retrospectively analyzed.Results The clinical courses of patients with intestinal Behcet's disease were from 26 days to 33 years,and the average duration was 6.32±1.01 years.The appearance of extra-gastrointestinal symptoms was significantly earlier than that of gastrointestinal symptoms(7.35±1.39 years vs.3.24±0.82 years,P<0.05).The predominant gastrointestinal manifestations were right lower quadrant pain(95.56%) and hematochezia or melena(40.00%).Misdiagnosis occurred in 17 cases.In patients without systemic medicine therapy before surgery,the incidence of postoperative infection of incision site and abdominal cavity was significantly higher than that in those undergoing systemic medicine therapy(80.00% vs.0%,P<0.05).Conclusions Because of the diversity of gastrointestinal manifestations,intestinal Behcet's disease is easily misdiagnosed.The systemic medical therapy before surgery could decrease the incidence of infection of incision and abdominal cavity.

Intestinal Behcet's disease with pyoderma gangrenosum:A case report

我们这里与脓皮病 gangrenosum 报导肠的 Behcet 的疾病的一个很稀罕的案例。被皮肤的过应性的存在和二个主要标准(口头、生殖器的 aphthoses ) 和一个次要的标准(胃肠的表明) 与肠的 Behcet 的疾病诊断的一个 16 岁的女人，被指我们有左的医院在 1989 年 9 月降低腿溃疡和腹的疼痛。结肠镜检查表明了包含全部冒号的骤发大肠炎。她的更低的腿损害是有不规则的边缘和一个破旧悬于边的痛苦的破坏溃疡。基于这些临床并且实验室调查结果，我们作为与加重的肠的 Behcet 的疾病发展的脓皮病 gangrenosum 诊断了她的皮肤的溃疡。尽管她接受了口头的氢化尼松处理一个月，她的皮肤、肠的损害糟糕被控制。因为有腹激怒的加重的腹的症状，我们在 1989 年 11 月执行了全部的结肠切除术。resected 标本与肠的 Behcet 与深溃疡和嗜中性的累积显示出严重发炎的疾病组织学地兼容。随后，脓皮病 gangrenosum 很快改善了。这堂临床的功课可以建议在脓皮病 gangrenosum 和肠的 Behcet 的疾病之间的靠近的关系。

Intestinal Behcet's disease with esophageal ulcers and colonic longitudinal ulcers

因为食道、回肠结肠的打外面的溃疡的周期性的口头的 aphthous，红斑象 nodosum 一样爆发，生殖器的溃疡，和内视镜的调查结果的历史，肠的 Behcet 在一个 38 岁的女人的疾病与结肠的纵的溃疡被诊断。食道的损害和结肠的纵的溃疡很少在肠的 Behcet 的疾病被看见。没有任何不利效果，食管和 ileocolon 的溃疡与氢化尼松和 mesalazine 与处理的 3 wk 愈合了。Mesalazine 可以减少氢化尼松的全部的剂量要求了治疗疾病。

H pylori infection in patients with Behcet's disease

AIM:To evaluate endoscopic findings and the prevalence of H pylori in patients with Behcet's disease (BD) who have upper gastrointestinal symptoms. METHODS:The patients with BD diagnosed according to the International Study Group and followed up in the Department of Dermatology and other related departments and who had any upper gastrointestinal complaints,were included in this study. Forty-five patients with BD and 40 patients in the control group were evaluated by upper gastrointestinal endoscopy and two biopsied specimens were taken during endoscopy for H pylori . A two-week triple therapy for H pylori eradication was administered to H pylori positive patients. Two months after the treatment,the patients were evaluated by urea-breath test for eradication control. RESULTS:Patients with BD had a mean age of 36.2 ± 11.4 years (18-67 years). The mean follow-up time was 35 ± 14 mo (16-84 mo). Aphthous or deep ulcer in esophagus,stomach and duodenum had never been confirmed by endoscopic examination. Most gastric lesions were gastric erosion (40%) and the most duodenal lesions were duodenitis (17.5%) in two groups. H pylori was positive in 33 patients (73.3%) with BD. The two-week triple eradication therapy was successful in 75% of the patients. There was no difference between the groups in respect to prevalence of H pylori (73.3% vs 75%,P > 0.05),and eradication rate (75% vs 70%,P > 0.05). CONCLUSION:Endoscopic findings,eradication rate and prevalence of H pylori were similar in patients withBD and control group.

A case of primary biliary cirrhosis complicated by Behcet's disease and palmoplantar pustulosis

一个 46 岁的女人在皮肤学的部门与掌跖脓疱病(PPP ) 被诊断，在 2000 的 Fukushima 医药大学医院，并且与软膏被对待。然而，因为肝机能障碍在 2003 发展了，她被指我们的部门，在主要胆汁性肝硬变(PBC ) 也根据临床的调查结果被诊断的地方。在 49 岁时，一年以后，她开发了 Behcet 的疾病(BD ) 的表明，包括在下肢的结节性红斑。因为她有眼色素层炎的历史，周期性的口头的溃疡是在场的，并且打字的 HLA 为 B51 是积极的， BD 另外被诊断。与 ursodesoxycholic (UDCA ) 在三月处理的开始以内使正常化的肝功能。这是与 BD 和 PPP 联系的 PBC 的第一个盒子。

Differentiation of Behcet's disease from inflammatory bowel diseases:Anti-saccharomyces cerevisiae antibody and anti-neutrophilic cytoplasmic antibody

The differential diagnosis of Behcet's disease(BD) from inflammatory bowel disease(IBD) is sometimes difficult and challenging.Hereby,we suggested the utility of anti-saccharomyces cerevisiae antibody(ASCA) and anti-neutrophilic cytoplasmic antibody(p-ANCA) in the differential diagnosis of BD from IBD.

Research Progress on TCM Treatment of Behcet's Disease

Behcet's disease is a vasculitis disease involving multiple systems,and its pathogenesis is still unclear.The paper discusses the research progress on the treatment of Behcet's disease in traditional Chinese medicine and integrated traditional Chinese and Western medicine.The clinical efficacy of integrated traditional Chinese and western medicine in the treatment of Behcet's disease is significantly better than that of Western medicine alone,which is worthy of further research.

Anti-TNF-αtreatment in Behcet's disease with cerebral vasculitis:a case report

27 44 岁的男病人与 Beh 被诊断 ? et 是疾病(BD ) 和以后的视觉小径爱(视觉外皮) ，它导致了视觉的减少的视觉尖酸和地。这个案例表明肿瘤的效果 Beh 上的坏死因素(TNF ) alpha blocker 治疗 ? et 的服的脉管炎。

The role of exosomes in adult neurogenesis:implications for neurodegenerative diseases

Exosomes are cup-shaped extracellular vesicles with a lipid bilayer that is approximately 30 to 200 nm in thickness.Exosomes are widely distributed in a range of body fluids,including urine,blood,milk,and saliva.Exosomes exert biological function by transporting factors between different cells and by regulating biological pathways in recipient cells.As an important form of intercellular communication,exosomes are increasingly being investigated due to their ability to transfer bioactive molecules such as lipids,proteins,mRNAs,and microRNAs between cells,and because they can regulate physiological and pathological processes in the central nervous system.Adult neurogenesis is a multistage process by which new neurons are generated and migrate to be integrated into existing neuronal circuits.In the adult brain,neurogenesis is mainly localized in two specialized niches:the subventricular zone adjacent to the lateral ventricles and the subgranular zone of the dentate gyrus.An increasing body of evidence indicates that adult neurogenesis is tightly controlled by environmental conditions with the niches.In recent studies,exosomes released from different sources of cells were shown to play an active role in regulating neurogenesis both in vitro and in vivo,thereby participating in the progression of neurodegenerative disorders in patients and in various disease models.Here,we provide a state-of-the-art synopsis of existing research that aimed to identify the diverse components of exosome cargoes and elucidate the therapeutic potential of exosomal contents in the regulation of neurogenesis in several neurodegenerative diseases.We emphasize that exosomal cargoes could serve as a potential biomarker to monitor functional neurogenesis in adults.In addition,exosomes can also be considered as a novel therapeutic approach to treat various neurodegenerative disorders by improving endogenous neurogenesis to mitigate neuronal loss in the central nervous system.

SIRT2 as a potential new therapeutic target for Alzheimer's disease

Alzheimer's disease is the most common cause of dementia globally with an increasing incidence over the years,bringing a heavy burden to individuals and society due to the lack of an effective treatment.In this context,sirtuin 2,the sirtuin with the highest expression in the brain,has emerged as a potential therapeutic target for neurodegenerative diseases.This review summarizes and discusses the complex roles of sirtuin 2 in different molecular mechanisms involved in Alzheimer's disease such as amyloid and tau pathology,microtubule stability,neuroinflammation,myelin formation,autophagy,and oxidative stress.The role of sirtuin 2 in all these processes highlights its potential implication in the etiology and development of Alzheimer's disease.However,its presence in different cell types and its enormous variety of substrates leads to apparently contra dictory conclusions when it comes to understanding its specific functions.Further studies in sirtuin 2 research with selective sirtuin2 modulators targeting specific sirtuin 2 substrates are necessary to clarify its specific functions under different conditions and to validate it as a novel pharmacological target.This will contribute to the development of new treatment strategies,not only for Alzheimer's disease but also for other neurodegenerative diseases.