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.

Assessment of Cardiac Autonomic Function by Using Heart Rate Turbulence in Behcet’s Disease

Behcet’s disease (BD) is a chronic inflammatory disorder that can affect many systems in the body. Cardiac involvement increases the risk of cardiovascular mortality and occurs in 1%-5% of patients with BD. Ventricular arrythmias are believed to be the cause of this increased risk of cardiovascular mortality and it is also thought to be related with cardiac autonomic dysfunction. Heart rate turbulence (HRT) is a new predictor of cardiac autonomic activity. HRT is an independent and powerful predictor of mortality. In this study, we investigated the cardiac autonomic activity which can be determined by HRT in patients with BD. Forty patients with BD (20 men, mean age: 40 ± 9 years, range: 27-55 years) were diagnosed according to the International Study Group Criteria (ISGC) and gender and age matched healthy volunteers (20 men, mean age: 39 ± 8 years, range: 26-56 years) were included in this study. All of the participants (patients and controls) underwent 24 hours Holter electrocardiogram. HRT parameters, turbulence onset (TO) and turbulence slope (TS) were calculated with HRT (View Version 0.60-0.1 of Software Program). There were no significant differences in TO and TS values between patients with BD and control subject (TO-BD: 0.014 ± 0.03, TO-Control: 0.011 ± 0.04;TS-BD: 7.88 ± 4.9, TS-Control: 9.42 ± 6.7 respectively). Although increased cardiovascular mortality rates in BD have been shown in many studies, HRT values—detecting the risk of sudden death—do not seem to be altered in this disease.

Bilateral pneumothorax and pneumomediastinum during colonoscopy in a patient with intestinal Behcet’s disease:A case report

BACKGROUND Colonoscopy is essential for the diagnosis of intestinal Behcet’s disease(BD),which is characterized by a typical oval-shaped ulcer in the ileocecal region.However,potential risks of colonoscopy have rarely been reported.CASE SUMMARY Herein,we describe a patient with intestinal BD who presented with decreased oxygen saturation and shortness of breath during a diagnostic colonoscopy.Bilateral pneumothorax,pneumomediastinum,pneumoperitoneum,pneumoretroperitoneum and subcutaneous emphysema of the neck,chest,abdomen,back and scrotum were confirmed by computed tomography scan.The sudden change in condition was considered to be associated with iatrogenic bowel perforation.After receiving closed thoracic drainage and conservative therapy,the patient was discharged in stable condition.CONCLUSION Endoscopists should be aware of the risks of colonoscopy in patients with intestinal BD and the possibility of pneumothorax associated with intestinal perforation and make adequate preparations before colonoscopy.

Amyloïdosis Complicating Behcet’s Disease

Background: Behcet’s disease is a vasculitis affecting several organs. A renal involvement is rarely described. It is most of the time about a renal amyloidosis. Patients and methods: It is a descriptive retrospective study concerning the patients followed in our department for a Behcet’s disease having presented a renal amyloidosis. Results: It is about 4 men with average age of 38.25 years old. The renal involvement was revealed after an average delay of 5.7 years by a nephrotic syndrome in all cases. A renal insufficiency was noted in 3 cases with an average serum creatinine of 587 μmol/l (127 - 1490). The type of the amyloidosis was AA in 2 cases. The treatment contained colchicines in every case. The evolution was marked by the worsening of the renal function leading to end stage renal disease in 3 cases. Death occurred in 1 case and one patient lost to follow up. Discussion: Renal amyloidosis can complicate the evolution of a Behcet’s disease. It occurs generally 1 to 10 years after the beginning of the disease. Once installed, it evolves generally towards the chronic renal insufficiency and can condition the forecast of this affection. Conclusion: Amyloidosis is a rare complication of the Behcet’s disease. Its screening is so desirable to improve the renal prognosis of these patients.

Evaluation of a new Tunisian version of behcet’s disease current activity form

Background: Beh&#231;et’s Syndrome (BS) is characterized by a heterogeneous vessel involvement, a fluctuating natural history and by the absence of biological markers correlated to disease activity that’s why objective clinical scores are needed for the assessment of its activity. The Beh&#231;et’s Disease Clinical Activity Form (BDCAF) is the most recent and widely used clinical activity score. Objectives: To perform a cross-cultural adaptation of the Beh&#231;et’s Disease Current Activity Form (BDCAF) to the Tunisian Dialect (Arabic Language) and to evaluate the metrological characteristics of the Tunisian version (Tu-BDCAF) especially its reliability in BD activity evaluation. Methods: Cross-cultural adaptation was done according to the established guidelines. Reliability of Tu-BDCAF was tested among 40 BD patients (mean age: 38 years, sex ratio: 1.37). Patients were questioned by two BD specialists at 20 minutes interval to evaluate inter-observer reproducibility and twice by the same physician at 48 hours interval to assess the intra-observer reproducibility. k Coefficient was used to test the concordance between qualitative variables and correlation between quantitative variables was evaluated used Pearson coefficient and Bland and Altman graphical method. Results: There was a good correlation between global scores calculated by the two physicians on the same day (r = 0.94, p < 0.0001) and also between the scores calculated by the same clinician at different times (r = 0.98, p k Coefficient analyses demonstrated a good intra and inter observer reliability for all the Tu-BDCAF items excepted for diarrhea and Clinician’s impression. As the original version, Tu-BDCAF is an objective, easy-calculated and reliable index for assessing disease activity in BD. The main limit of the BDCAF score remains the absence of a cut-off point defining BD activity. Conclusion: Tu-BDACF is a Tunisian version of the BDCAF score which can be used in routine to assess BD activity but also in international studies and clinical trials.

Biologic Agents in Behcet’s Disease:Our Experience and Review of the Literature

Behcet’s disease (BD) is a large vessel vasculitis with a wide range of clinical manifestations. Some of these manifestation may be life threatening and rapid suppression of the inflammation with effective immunosuppressive agent is crucial. There are traditional drugs with different response rates and all have efficacy on different manifestations of the disease. The most frightening manifestations of the disease are ocular, neurologic, intestinal and vascular types of involvement. Besides benign and easily treated manifestations there are also refractory cases with complicated involvement. The novel biologic agents have been used for these resistant patients and favorable response rates have been reported. In this review, we have shared our experience with biologic agents in BD and also reviewed the literature for the efficacy and safety for these novel agents for refractory patients.

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.

Effects of anti-TNF biologic drugs on uveitis severity in Behcet patients: systematic review and Meta-analysis

AIM:To investigate effects of anti-TNF biologic drugs on uveitis severity(comparing visual acuity log MAR levels) in Behcet patients.METHODS:Three databases Pub Med, Scopus, and the Web of Science were searched for qualified papers focusing on the anti-TNF-α factors treatment in Behcet’s disease(BD)-associated uveitis. Studies that were designed pre and post anti-TNF drug treatment, were selected. After determining the search strategy for this study, the relevant data were extracted. RESULTS:The initial search was performed in the target databases and a total of about 1458 articles were found. Fifteen articles were selected for systematic review and only 12 of them had inclusion criteria for Meta-analysis(with visual acuity data). The mean dose of prednisolone before and after biological treatments was reported in 5 studies(28.56 and 7.56 mg/kg, respectively). Also, the preliminary results indicate a significant reduction in visual acuity log MAR levels(MD=-1.5 IU/L, 95%CI:-2.1,-0.01).CONCLUSION:Biological drugs significantly reduce the dose of prednisolone and affect visual acuity values.

Endoscopic and radiographic features of gastrointestinal involvement in vasculitis

Vasculitis is an inflammation of vessel walls,followed by alteration of the blood flow and damage to the dependent organ.Vasculitis can cause local or diffuse pathologic changes in the gastrointestinal (GI) tract.The variety of GI lesions includes ulcer,submucosal edema,hemorrhage,paralytic ileus,mesenteric ischemia,bowel obstruction,and life-threatening perforation.The endoscopic and radiographic features of GI involvement in vasculitisare reviewed with the emphasis on small-vessel vasculitis by presenting our typicalcases,including Churg-Strauss syndrome,HenochSch nlein purpura,systemic lupus erythematosus,and Beh et's disease.Important endoscopic features are ischemic enterocolitis and ulcer.Characteristic computed tomographic findings include bowel wall thickening with the target sign and engorgement of mesenteric vessels with comb sign.Knowledge of endoscopic and radiographic GI manifestations can help make an early diagnosis and establish treatment strategy.

Surgical Intervention for Behcet＇s Disease with Aorta Aneurysm and Pseudoaneurysm： Opposite Outcomes in Two Cases

Behcet＇s disease （BD） is a chronic, relapsing autoimmune disorder characterized by oral and genital ulcerations with uveitis, and additional clinical manifestations in multiple organ systems. The occurrence of vascular involvement in BD is reported to be in the range of 5 30%.

The genetics of Behcet’s disease in a Chinese population

Behcet’s disease is defined as a multisystemic inflammatory disease.Although the precise pathogenesis and etiology is still a mystery,accumulating evidence shows that genetic variants of immune-related genes have a profound influence on the development of Behcet’s disease.To explore the genetic factors for Behcet’s disease,our group investigated the association of Behcet’s disease with multiple immune response genes and has identified multiple Behcet’s disease-related immunoregulatory pathways in the Chinese Han population.A large number of gene polymorphisms were studied including STAT4,IL23R,CD40,CCR1/CCR3,STAT3,OPN,IL17,JAK2,MCP-1,CTLA4,PD-1,PD-L1,PD-L2,TGRBR3,CCR6,PTPN22,FCRL3,IRF5,SUMO4 and UBAC2.Significant associations were found between Behcet’s disease and STAT4,IL23R,CD40,CCR1/CCR3,STAT3,MCP-1,TGFBR3,FCRL3,SUMO4,UBAC2.These genetic predisposition studies support an important role for both lymphocyte differentiation as well as ubiquitination pathways.These findings are helpful in elucidating the pathogenesis of Behcet’s disease and hopefully will allow the development of novel treatment regimes.

Chinese Medicine in the Treatment of Behcet's Disease's Uveitis:A Case Report

Introduction Behcet＇s disease（BD） is an multi-system inflammatory vasculitis based on small and large vessels of the venous and arterial system.About 80%of BD patients have ocular lesions characterized by recurrent iridocyclitis,retinal vasculitis,and retinal ischemia.Frequent recurrence of these lesions,leading to macular edema and optic atrophy,can eventually result in severe loss of vision.Western medicine treatment focuses on steroid and immunosuppressive agents;

Urological manifestations and treatment of the primary systemic vasculitides

The primary systemic vasculitides(PSV) are a group of rare inflammatory disorders affecting blood vessels of varying size and multiple organs. Urological manifestations of PSV are uncommon. Testicular vasculitis is the most commonly reported finding and is associated with Polyarteritis Nodosa(PAN), Henoch-Sch?nleinPurpura(HSP), anti-neutrophil cytoplasm antibody associated Vasculitides(AAV), Giant Cell Arteritis(GCA) and Kawasaki disease. Prostatic vasculitis has been reported in association with GCA and AAV. Ureteric involvement has been noted in PAN, HSP and AAV. Other urogenital manifestations of PSV include genital ulceration and bladder dysfunction in Beh?ets Disease and haematuria which is commonly seen in many of the PSV. Finally, therapies used to treat the PSV, especially cyclophosphamide, are associated with urological sideeffects including haemorrhagic cystitis and urothelial malignancy. The aim of this review is to examine how the urological system is involved in the PSV. Each PSV is examined in turn, with a brief clinical description of the disease followed by a description of the urological manifestations and management. Identification of urological manifestations of PSV is important as in many cases symptoms may improve with immunosuppressive therapy, avoiding the need for invasive surgery. Additionally, patients who present with isolated urogenital PSV are at higher risk of developing subsequent systemic vasculitis and will need to be followed up closely.

Modified Bentall procedure for aortic prosthetic detachment due to Behcet's disease

Background Severe aortic regurgitation is a rare clinical presentation of Behcet＇s disease,but the valve detachment after valve replacement presents challenges to cardiac surgeons.Methods Five patients with prosthetic detachment after aortic valve replacement attributable to Behcet＇s disease were surgically treated between January 2007 and December 2010.These 5 patients （all male） were from 24 to 50 years of age （mean,38.8 ± 9.8）.Mean follow-up duration from index operations was 19.2 ± 12.5 months （range,3-36 months）.Results There was no early death and pseudoaneurysm of the aortic root occurred at index operations.But one patient suffered Ⅲ° AVB and had pacemaker implanted.All the patients were administered immunosuppressive therapies after operation.Conclusion Modified Bentall operation and postoperative immunosuppressive therapy may be helpful for the patients with valve detachment after valve surgery due to Behcet＇s disease.

Outcomes of Open Surgery for Popliteal Artery Aneurysms:An 18-Year Experience in a Single Institution in Japan

Objective: To elucidate the outcomes of open surgery for popliteal artery aneurysm (PAA) in a single Japanese institution. Materials and Methods: Between 1999 and 2016, 35 patients (44 PAAs) underwent open surgery in our department. The first-line surgical strategy involved the medial approach, with excision of the aneurysm sac and autologous vein bypass grafting. Associations between the outcomes and 1) the surgical management of the aneurysmal sac (aneurysmectomy vs. ligation with bypass) and 2) the preoperative clinical symptoms were evaluated. Results: There were no aneurysm-related deaths during the follow-up period (mean, 4 years;range, 1 - 17.6 years). The 5-year primary, primary-assisted, and secondary patency rates were 67.9%, 72.7%, and 94.5%, respectively. Re-intervention was required for 19 PAAs (14 patients), including two PAAs in patients with suspected Beh&ccedil;et’s disease. Patency rates did not differ between the aneurysmectomy and ligation groups. Remnant sac enlargement occurred in 3 cases. Among asymptomatic patients, clinical symptoms did not deteriorate;however, the ankle brachial pressure index decreased ≥0.15 in patients with artery-graft mismatching. Conclusion: Patency rates were good, and our modified ligation with bypass procedure, which involves excising the sac as much as possible, exhibited less risk in terms of remnant sac enlargement compared to that in previous studies.

Discriminating Potential of Extraintestinal Systemic Manifestations and Colonoscopic Features in Chinese Patients with Intestinal Beh~：et＇s Disease and Crohn＇s Disease

Background：The distinction between intestinal Beh（c）et＆#39;s disease （BD） and Crohn＆#39;s disease （CD） is always challenging due to many overlapping clinical features.We conducted a retrospective study to reveal valuable strategies for the differential diagnosis between intestinal BD and CD in Chinese patients based on their clinical and colonoscopic features.Methods：Thirty-five intestinal BD patients and 106 CD patients hospitalized from January 1983 to January 2010,who had ulcerative lesions in the terminal ileum or colon under colonoscopy and no history of gastrointestinal operation except appendectomy before admission,were enrolled.Univariate and multivariate logistic regression analyses were conducted to find discriminating predictors among demographic data,clinical manifestations,and colonoscopic findings.Results：Based on univariate analysis,massive gastrointestinal hemorrhage,fever,and extraintestinal systemic manifestations were more common in intestinal BD patients （P =0.022,0.048 and 0.001,respectively）,while diarrhea,intestinal obstruction,and perianal lesions were more common in CD patients （P =0.002,0.010,and 0.027 respectively）.Based on colonoscopy,focal involvement,ileocecal valve deformity,solitary ulcers,large ulcers （ulcer size 〉 2 cm）,and circumferential ulcers were more common in intestinal BD patients （P =0.003,0.003,0.014,0,013,and 0.003,respectively）,while segmental involvement,longitudinal ulcers,a cobblestone or nodular appearance,and pseudo-polyps were more common in CD patients （P =0.003,0.008,0.023,and 0.002,respectively）.Based on multivariate logistic regression analysis,diarrhea,extraintestinal manifestations,ulcer distribution,size,and type,and pseudo-polyps were independent discriminating predictors between the two groups （P =0.048,0.008,0.006,0.021,0.002,and 0.041,respectively）.The discriminating algorithm composed of the above independent predictors had the highest area under the curve of 0.987 for distinguishing between the two diseases.Conclusions：Extraintestinal systemic manifestations and the characteristic colonoscopic features,such as ulcer distribution,size and type,helped to distinguish intestinal BD from CD.