Behcet's Disease Research Articles

Prevalence, types, and characteristics of headache in Behcets disease without involvement of the central nervous system in the Syrian population: a case-cohort study.

Oral ulcers, genital ulcers, and uveitis represent the typical trial of Behcet's disease (BD). It is well common on the Old Silk Road. The mucocutaneous lesions are the hallmark of BD, but neurological involvement is one of the severe symptoms. Headaches may be an early sign of BD neurological involvement. This study aims to investigate the headache prevalence and its types, and characteristics in a Syrian sample of BD patients. BD patients were clinically interviewed and examined to collect their information, symptoms, and signs. the International Study Group for Behcet's Disease diagnosis criteria was used to confirm the BD diagnosis. The International Classification of headaches was used when classifying the headaches. One hundred twenty participants were included in the study. half of them were BD patients and the control group was also 60 participants. Among Syrian BD patients, 36.7% suffer from primary headaches and 36.7% suffer from secondary headaches. These findings were not significantly different between the BD patients and the healthy population. Our results showed that there was no statistically significant difference between the two groups. Headache should not be considered a predictor for neurological involvement among BD patients. Additional attention to BD patients or the specific treatment for headaches is not required and does not differ from the general population.

An investigation of the relationship between Behçet's disease and tenascin-C.

The study aimed to investigate serum tenascin-C levels and its relationship with pathogenesis of Behçet's disease (BD) with inflammatory processes. This prospective and analytical study included 34 BD patients (19 males, 15 females; mean age: 31.5±8.2 years; range, 18 to 48 years) who met the 2014 International Criteria for Behçet's Disease and had no comorbidities and 37 healthy volunteers (21 females, 16 males; mean age: 29.6±5.3 years; range, 21 to 45 years). Sex, age, age at diagnosis, clinical and laboratory data, medication use, and smoking history of the participants were recorded. Serum tenascin-C levels were measured using a commercially available tenascin-C enzyme-linked immunosorbent assay kit. There was no significant difference between the groups in terms of age (p=0.262) and sex (p=0.287). Serum tenascin-C levels were significantly lower in the BD group (10,824±7,612 pg/mL) compared to the control group (27,574±14,533 pg/mL, p<0.001). In the receiver operating characteristic analysis performed for the diagnostic value of tenascin-C level in BD, the sensitivity was determined as 79.4% and the specificity as 82.5% (p<0.001). No statistically significant difference was observed in tenascin-C levels in correlation with clinical characteristics, laboratory values, medication use, and smoking in the BD group. In contrast to other chronic inflammatory diseases, lower levels of tenascin-C were observed in patients with BD than in the healthy individuals, which can be attributed to the absence of prolonged chronic inflammatory course in BD. The fact that tenascin-C levels are high in other rheumatic inflammatory diseases but low in BD may be useful in the differential diagnosis of BD.

Neurological and Vascular Behçet's Disease in a Young Male without Classic Triad of Behçet's Disease: A Case Report and Literature Review.

Neuro-Behçet's disease (NBD) is an uncommon presentation in Behçet's disease (BD) with severe course and worse prognosis. Both vascular and NBD presentation without the classical triad of BD in a single patient is rarely reported. Here a 48-year-old male had an extensive aortic aneurysm eroding vertebra for which he was diagnosed as vascular BD. Two years later, he was presented with a severe headache and cerebrovascular accident, his brain imaging showed hyperintensity in the right thalamus, basal ganglia, temporal lobe, and internal capsule, suggesting the 'cascade sign' of NBD. Surprisingly, he never had oral or genital ulcers or skin and eye involvement. He had a good response to infliximab. Clustering of BD phenotype is an emerging area of interest. It is hypothesised that severe phenotype of vascular and parenchymal NBD can happen in the same patient owing to similar underlying pathology. This case is unique due to its severe phenotype with no features of the typical triad of BD.

Does anticoagulation in combination with immunosuppressive therapy prevent recurrent thrombosis in Behçet's disease?

Vascular involvement in Behçet's disease (BD) occurs in up to 50% of patients. The main mechanism of thrombosis is inflammation. Thus, immunosuppressants (IS) are the mainstay of therapy, and adding anticoagulation (AC) is controversial. In daily practice, we observed that patients who received AC in combination with IS experienced less recurrent thrombosis and decided to investigate our BD patients retrospectively. We hypothesized that adding AC to immunosuppressive therapy may lower the risk of recurrent thrombosis. Treatment at the time of first or recurrent thrombotic events was recorded. Events under the only IS and IS + AC treatments were compared. There were 40 patients (33 males). The most common types of first vascular events were deep vein thrombosis (77.5%) followed by pulmonary embolism (PE) (52.5%). One patient did not receive any treatment. Among the 39 patients, 32 received glucocorticoid and at least one of the azathioprine, or cyclophosphamide, anti-TNF, 5 received monotherapy with azathioprine, 1 received monotherapy with corticosteroid, and the remaining 1 received monotherapy with cyclophosphamide. In total, 22 patients (55%) experienced 27 recurrent venous thromboembolism (VTE) events. Two (7.4%) events while only on AC, 2 (7.4%) events while on AC + IS, and 15 (55.5%) events occurred while on only IS. Eight (19.6%) patients were not receiving any treatment during relapses. The recurrence rate was statistically significantly lower in the IS + AC treatment group compared to IS alone. In conclusion, IS are the mainstay of treatment for BD, and adding AC may help to lower the recurrence risk of thrombotic events.

Short- and long-term outcomes of surgical treatment in patients with intestinal Behcet's disease.

Behcet's disease (BD), a chronic vasculitic disorder affecting multiple organs, is characterized by recurrent oral and genital ulcers, arthritis, vasculitis, and intestinal ulcers. Although intestinal involvement of BD is common in East Asia, the efficacy and long-term outcomes of surgical treatment of intestinal BD still remain to be established. To evaluate the postoperative clinical course of intestinal BD and determine factors associated with its recurrence. Data from patients who underwent surgical treatment for intestinal BD between January 2010 and August 2021 were retrospectively reviewed. Patients' demographics, clinical features, postoperative course, complications, and follow-up data were evaluated. We analyzed 39 surgeries in 31 patients. The mean patient age was 45.1 years, and the mean interval between the diagnosis of intestinal BD and surgical treatment was 4.9 years (range 1.0-8.0 years). The most common indication for surgery was medical intractability (n = 16, 41.0%), followed by fistula or abscess (n = 11, 28.2%). Laparoscopic approaches were used in 19 patients (48.7%), and 5 patients (12.8%) underwent emergency surgeries. The most common surgical procedure was ileocecal resection (n = 18, 46.2%), followed by right colectomy (n = 11, 28.2%). A diverting stoma was created in only one patient (2.6%). During a mean follow-up period of 45 (range 8-72) months, eight cases (20.5%) of recurrence in five patients required reoperation. The interval between operations was 12.1 months (range 6.3-17.8 mo). Four patients (10.3%) experienced recurrence within 1 year postoperatively, and all eight recurrences occurred within 2 years of the initial surgery. The reoperation rates at 1 and 3 years were 10.3% and 20.5%, respectively. A redo ileocolic anastomosis was performed in all recurrent cases. In multivariate Cox regression analysis, emergency surgery [hazard ratio (HR) 9.357, 95% confidence interval (CI): 1.608-54.453, P = 0.013] and elevated C-reactive protein (CRP) levels (HR 1.154, 95%CI: 1.002-1.328, P = 0.047), but not medication use, were predictors of recurrence. Surgical resection is a feasible treatment option for complicated BD. Reoperation is associated with severe inflammatory conditions, reflected by increased CRP levels and the requirement for emergency surgery.

Neither hepatic steatosis nor fibrosis is associated with clinical outcomes in patients with intestinal Behçet's disease.

Behçet's disease (BD) and nonalcoholic fatty liver disease (NAFLD) are chronic inflammatory diseases that share pathogenetic mechanisms. In this study, we investigated whether NAFLD influences the clinical outcomes in patients with intestinal BD. Patients with intestinal BD and available hepatic steatosis index (HSI) and fibrosis-4 (FIB-4) scores were recruited between 2005 and 2022. An HSI of ≥30 and FIB-4 of ≥1.45 were used to diagnose hepatic steatosis and significant liver fibrosis, respectively. The primary outcomes were intestinal BD-related hospitalization, surgery, emergency room visits, or the first use of corticosteroids, immunomodulators, or biologic agents for intestinal BD. A total of 780 patients with BD were selected. The prevalence of hepatic steatosis and significant liver fibrosis were 72.3% and 8.8%, respectively. Multivariate analysis showed that younger age, prior smoking history, concomitant skin lesions, higher white blood cell count, and lower serum albumin levels were independently associated with an increased risk of clinical relapse (all P < 0.05), whereas hepatic steatosis and significant liver fibrosis were not (hazard ratio [HR] = 1.164, 95% confidence interval [CI] 0.923-1.468; P = 0.199 for hepatic steatosis; HR = 0.982, 95% CI 0.672-1.436; P = 0.927 for significant liver fibrosis). Hepatic steatosis and liver fibrotic burden were not independently associated with clinical outcomes in patients with intestinal BD.

Increased inferior vena cava wall thickness as a sign of extensive venous inflammation in Behçet's Disease.

Behçet's disease (BD) affects both arterial and venous vessels. We have previously shown that common femoral vein wall thickness (WT) is increased in BD and can be used as a diagnostic test. However, there is limited data assessing large veins. Therefore, this study seeks to assess inferior vena cava wall thickness (IVC) by transthoracic echocardiography (TTE) in BD compared to healthy controls (HC). Age- and gender-matched 70 BD patients and 51 HC were included. IVC wall thickness and common femoral vein WT were measured by TTE and Doppler ultrasonography, respectively. All examinations were performed on the same day as the clinical assessment. The mean IVC wall thickness of BD patients was significantly higher than HC (2.9mm (0.3) vs 2.6mm (0.3), p < 0.001). Patients with mucocutaneous involvement (2.8mm (0.3)) and major organ involvement (2.9mm (0.3)) had significantly thicker walls compared to HC (p = 0.003, p < 0.001, respectively). IVC wall thickness was higher in patients with vascular involvement compared to those with nonvascular major organ involvement (3.1mm (0.3) vs 2.8mm (0.2), p = 0.04). There was a moderate correlation between IVC and common femoral vein WT (r = 0.49 for the right, r = 0.43 for the left, p = 0.01 for both). This study shows that venous wall inflammation is not limited to lower extremity veins and is also present in IVC walls of BD patients regardless of IVC involvement. Vascular wall inflammation is probably a widespread vascular event of all venous walls in BD. Key Points • Venous wall inflammation is not limited to lower extremity veins and is present also in IVC wall in Behçet's disease. • Extensive venous wall inflammation in Behçet's disease includes large venous structures despite not being clinically involved.

Common femoral vein wall thickness measurement by Doppler ultrasonography is an accurate diagnostic test for Behçet's Disease both in supine and standing positions.

We recently reported the first controlled Doppler ultrasonography (US) study demonstrating increased common femoral vein (CFV) thickness in Behçet's Disease (BD). Standard lower extremity venous Doppler US is performed in erect position. In order to confirm accuracy and applicability of method, we measured CFV thickness in both supine and standing positions in this study. We included sex and age-matched 42 BD patients and 41 healthy controls (HCs). After routine visits, bilateral CFV thickness was measured with Doppler US both in supine and standing positions. Bilateral CFV thickness was significantly higher in BD than in HC. There were no statistically significant differences in measurements of CFV wall thickness between standing and supine positions in both groups. CFV measurement by Doppler US is a new and non-invasive diagnostic tool for the diagnosis of BD. Our study confirmed that patient position does not affect CFV wall thickness measurement for diagnosis of BD.

Behçet Hastalarında Kardiyak Tutulum: Retrospektif Tek Merkez Deneyimi

Behcet’s disease (BD) is well-known with mucocutaneous involvement, whereas the heart may rarely be involved, predicting morbidity and mortality. In this study, we aimed to reveal the clinical characteristics of cardiac involvement in BD. We retrospectively screened 800 BD patients diagnosed between 2000 and 2021 for cardiac involvement. 14 patients who met these criteria were recruited in this study. Demographic information, clinical features, treatment modalities, and prognosis were evaluated. All patients were male and smokers. The mean age for cardiac involvement was estimated at 32.5 ±7,8 years. Cardiac involvement developed in one-third of the patients before disease diagnosis. Patients were classified into three major groups: 8 of intracardiac thrombosis (ICT), 2 of coronary artery aneurysms, and 4 of myocardial infarction (MI). The majority of ICT was seen in the right ventricle (6 out of 8). In all MI cases, the left main coronary artery was totally occluded. Deep vein thrombosis was seen in 57% of patients. Apart from steroids, cyclophosphamide was the most common preferred agent, used in 9 patients. Azathioprine and interferon use were seen in 4 cases. Warfarin was used in 10 patients and 4 cases received an antithrombotic agent. Mortality was seen in 2 cases due to unknown causes. Cardiac involvement is rare, but a serious manifestation of BD. ICT was the most common type with mostly involved the right heart chambers. Male gender and smoking were found as the most important associated risk factors in this population.

Effect of adalimumab on choroidal thickness and choroidal vascularity index in eyes with non-infectious uveitis using enhanced-depth imaging optical coherence tomography

ObjectiveTo evaluate the effect of adalimumab (ADA) on choroidal thickness (ChT) and choroidal vascularity index (CVI) in eyes with non-infectious uveitis (NIU).MethodsThirty-seven eyes with NIU including Behçet disease (BD), sarcoidosis, ankylosing spondylitis (AS), juvenile idiopathic arthritis and idiopathic arthritis, 38 eyes of non-uveitic (NU) patients including BD, AS and rheumatoid arthritis, and 40 healthy control eyes were included. ADA was used for anti-TNF-naive adult (80 mg) or paediatric (40 mg) patients with refractory NIU, then 40 mg every 2-week (20 mg in children<30 kg) with controls at weeks 1, 4, 12, and 24. Images were used to measure central, nasal, and temporal ChT, and the luminal area (LA), stromal area, and total choroidal area (TCA) were analysed using enhanced-depth imaging optical coherence tomography (EDI-OCT) by ImageJ software. The CVI was then calculated as the ratio of LA to TCA.ResultsMean ages were similar between the groups. Mean (SE) subfoveal ChT measurements for each location were also similar (for each, p > 0.05). However, calculated CVI values in eyes with NIU (0.63 ± 0.007) were significantly (p < 0.001) lower than NU eyes (0.66 ± 0.006) and controls (0.70 ± 0.007) (p < 0.001). Moreover, CVI was significantly lower in NU eyes compared to controls (p < 0.001). There were no significant CVI changes between the consecutive visits after ADA therapy in eyes with NIU (for each, p > 0.05).ConclusionsDecreased CVI in NIU and NU eyes indicates that systemic inflammation affects the choroidal vasculature and perfusion both in the presence and absence of ocular involvement. Although CVI may be used as a possible novel tool in monitoring ocular involvement and progression of NIU, CVI does not seem to be a biomarker for treatment monitoring in NIU.

Inflammatory and neutrophil activation markers in Behcet's disease

Behcet's disease (BD) is a systemic vasculitis characterized by recurrent episodes of inflammation with aphthous stomatitis, genital ulcers, skin, joint and internal organ involvement. Currently, there are no reliable laboratory markers that can be used to monitor BD activity. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) have low sensitivity, so the search for new biomarkers continues. Neutrophil reactivity intensity (NEUT-RI), neutrophil granularity intensity (NEUT-GI), immature granulocytes (IG), neutrophil-to-lymphocyte ratio (NLR), systemic immune inflammation index (SII) are new inflammatory indicators whose role in BD is not well studied.Objective: to evaluate and compare the efficacy of the determination of NEUT-RI, NEUT-GI, IG, SII, NLR, ESR and CRP in the diagnosis of active BD.Material and methods. 84 patients with a reliable diagnosis of BD and 38 healthy controls were included in the study. BD activity was assessed using the Behcet’s Disease Current Activity Form (BDCAF). Patients with BD were divided into two groups according to activity: Group I included 41 patients with high activity (BDCAF &gt;5) and Group II included 43 patients with low activity (BDCAF ≤5). A complete blood count with determination of NEUT-RI, NEUT-GI, IG, SII and NLR was performed in all patients and healthy donors using the Sysmex XN 1000 automated haematology analyzer (Sysmex Сorp, Japan). ESR was determined using the Westergren method. High-sensitivity serum CRP level was determined in all patients with BD (normal range ≤5) by immunonephelometric method.Results and discussion. Patients with active BD had significantly higher levels of neutrophils, SII, NLR, NEUT-RI, IG, ESR and CRP compared to patients with low disease activity. ROC analysis was performed to compare the significance of these inflammatory markers in the assessment of BD activity. The SII had the largest area under the ROC curve (AUC =0.816). The sensitivity and specificity of the SII at a value of 509.75 or higher for determining the active form of BD were 79.4% and 71.8%, respectively.Conclusion. The new inflammatory parameters (SII, NLR, NEUT-RI and IG) and the traditional inflammatory parameters (ESR, CRP, neutrophils) can serve as laboratory markers for BD activity. SII is the most informative parameter to determine BD activity with optimal sensitivity and specificity.

Tacrolimus-Associated Terminal Ileitis After Kidney Transplantation, Mimicking Crohn Disease: A Case Report

The onset of gastroduodenal ulcers is a frequent complication after transplantation, whereas cases of intestinal ulcers are sporadic and poorly described in the literature. A patient on immunosuppressive therapy with tacrolimus and mycophenolate mofetil after kidney transplant for immunoglobulin A-related glomerulonephritis developed symptoms compatible with Crohn disease 7 months after the transplant. The patient was hospitalized for abdominal pain, diarrhea, fever, and weight loss. Imaging and a colonoscopy showed signs of idiopathic inflammatory bowel disease (IBD) affecting the terminal ileum. Behcet's disease, post-transplant lymphoma, cytomegalovirus, Epstein-Barr virus, or mycobacteria infection were excluded. Mycophenolate mofetil was suspended, and steroid therapy was increased without clinical improvement. Eleven units of blood were required for severe anemia. A further colonoscopy revealed ulcerations involving the cecal fundus, ileocecal valve, and distal ileum with bowel stenosis and suspected ischemia. The patient, therefore, underwent an emergency laparoscopic ileocolic resection. The histologic examination did not reveal clear signs of IBD, ischemia, or viral infection of the ileum. The findings seemed indicative of iatrogenic damage from immunosuppressive therapy. The postoperative course was regular, and after 12 months, the patient was asymptomatic, on low-dose tacrolimus and prednisone therapy. During immunosuppressive therapy, the onset of isolated ileal ulcers, which can mimic IBD, may be a sporadic complication.

Effect of Infliximab on Chronic Progressive Neuro-Behçet's Disease: Influence of the Timing of Introduction on the Patient Outcome.

Objectives Chronic progressive neuro-Behcet's disease (CPNB) is characterized by progressive deterioration leading to disability. Methotrexate (MTX) has been shown to have beneficial effects on CPNB. However, while infliximab has been found to be effective for patients with inadequate responses to MTX, the appropriate timing for the introduction of infliximab remains unclear. We explored the effects of intervals before the introduction of infliximab on the functional outcome. Methods A retrospective analysis was performed for patients with CPNB who received infliximab and were followed up until October 2015. Functional disability was rated by the Steinbrocker functional classification as used in rheumatoid arthritis. Correlations between the outcomes and intervals before the introduction of infliximab were then analyzed by Spearman's rank correlation test. Patients Eleven patients with CPNB [8 men, 3 women, age 35.2±9.3 years old (mean±standard deviation)] who met the international classification criteria for Behcet's disease were included. Results All 11 patients had received MTX prior to infliximab. The intervals from the onset to the introduction of infliximab and the follow-up periods were 26.6±35.1 months and 65.2±43.6 months [mean±standard deviation], respectively. Among the 11 patients, 2 still showed progression after the introduction of infliximab. The functional disability grades after infliximab treatment were significantly correlated with the intervals from the onset of CPNB to the introduction of infliximab (r=0.6177, p=0.0476). Conclusion The results indicate that the delayed introduction of infliximab leads to irreversible functional disability in CPNB. Thus, it is recommended that infliximab be administered as soon as possible for CPNB patients with inadequate responses to MTX.

A166 STEROID-REFRACTORY ILEITIS FOLLOWING RITUXIMAB THERAPY: A CASE REPORT AND LITERATURE REVIEW

Abstract Background Rituximab, an anti-CD20 monoclonal antibody commonly used in treatment of hematologic and rheumatologic disease is generally well-tolerated from a gastrointestinal perspective however rare reported side effects include diarrhea and enterocolitis. Aims We describe a case of steroid-refractory ileitis presenting in a woman with follicular lymphoma post Rituximab therapy. Methods A case report and literature review were performed. Results A 73-year-old woman presented with a 4-week history of fevers, bloody diarrhea and abdominal pain. She had a known history of stage IV follicular lymphoma treated with six cycles of Bendamustine and Rituximab followed by four doses of maintenance Rituximab, with the last dose ending 4 weeks prior to her presentation. On examination, she had multiple peri-anal round ulcers. An abdominal CT revealed circumferential mural thickening involving the distal terminal ileum. On colonoscopy, there were multiple discrete ulcers and nodularity in the terminal ileum. Pathology showed evidence of chronic active ileitis with no signs of malignancy, lymphoproliferative disorders, or granulomas. AFB smear and cultures were negative. She was given IV steroids for 1 week and then placed on a Prednisone taper scheduled to finish in 9 weeks. 4 weeks later she presented again with fevers and abdominal pain. Inflammatory markers were elevated, and a repeat CT abdomen showed ongoing multifocal ileitis. She was put back on IV steroids and monitored. She continued to have persistent abdominal pain and fevers, and then underwent a repeat colonoscopy after 9 days of IV steroids which showed an interval increase in the size of her ileal ulcers. Pathology showed granulation tissue and reactive changes with no evidence of malignancy, lymphoma, granulomas, inclusion bodies or other viral cytopathic effects. Repeat AFB smear was negative. CMV PCR in blood was negative. She was assessed for Behcet’s disease (BD) given her peri-anal ulcers however she lacked other findings needed to fulfill the diagnostic criteria for BD. Steroids were continued with plans to initiate biological therapy. A review of literature revealed rare reports of rituximab-associated ileocolitis and development of de novo inflammatory bowel disease post rituximab therapy. Among patients who received Rituximab and underwent Colonoscopy, 4% had evidence of colitis. Rituximab-associated colitis appears to be usually mild presenting with diarrhea and requiring supportive care only, but in rare cases can lead to fulminant colitis and bowel perforation. Conclusions Rituximab-associated enterocolitis remains an under-reported entity and can lead to significant morbidity and treatment-associated challenges requiring prolonged courses of steroids and considerations for biological therapies. Important differentials to consider include malignancy, lymphoma, Tuberculosis, Bechet’s disease, and CMV enterocolitis. Ulcer in terminal ileum and ileocecal valve - Pre steroids (Left), Post steroids (Right) Funding Agencies None

Investigation of optic nerve function in Behçet's patients with optic disc hyperfluorescence in fundus fluorescein angiography.

The aim was to investigate the changes in optic nerve function that may help in the diagnosis of subclinical optic nerve involvement in patients with Behçet's disease (BD) and isolated optic disc (OD) hyperfluorescence in fluorescein angiography (FA). Three groups were formed; BD patients with isolated OD hyperfluorescence in FA, BD patients without ocular involvement (normal FA) and control group. A total of 88 eyes of 45 patients were included. The groups were compared in terms of OCT-RNFL, contrast sensitivity and VEP latency. When the OCT-RNFL values were compared, there was a significant difference between the control group and Behçet's patients with normal FA. Contrast sensitivity values differed significantly among the groups, and the lowest mean contrast sensitivity was observed in the group with OD hyperfluorescence (p < 0.05). As far as we know, this is the first publication that investigates optic nerve function in BD patients with isolated OD hyperfluorescence in FA. Assessment with FA of asymptomatic BD patients with visual complaints and low contrast sensitivity may be helpful at early detection of inflammatory optic neuropathy by close follow-up in patients with OD hyperfluorescence.

All-cause and cause-specific mortality in patients with Behçet disease versus the general population.

The comparative risk of cause-specific mortality in patients with Behçet disease (BD) vs. the general population is not known. To compare the risk of all-cause and cause-specific mortality in patients with BD vs. the general population. Using data from the Korea National Health Insurance Service database for the period 2002-20, we conducted a cohort study comparing patients with BD with the general population, matched according to age and sex (1 : 4 ratio). We used Cox proportional hazard models to estimate hazard ratios (HRs) and 95% confidence intervals (CIs) for all-cause and cause-specific mortality. Subgroup analyses by age and sex were done. We included 24 669 patients with BD and 98 676 age- and sex-matched controls [mean (SD) age 40.5 (12.9) years; 34% male]. During a mean follow-up of 11.9 years, the incidence rate (IR) of death per 100 person-years was 0.36 in patients with BD and 0.29 in controls [hazard ratio (HR) 1.28, 95% confidence interval (CI) 1.20-1.38]. The risk of mortality was highest in the first year after BD diagnosis (HR 2.66, 95% CI 2.09-3.40). Patients with BD died more often in this period as a result of malignancy (HR 1.96, 95% CI 1.30-2.98); cardiovascular (HR 2.68, 95% CI 1.45-4.97), gastrointestinal (HR 3.50, 95% CI 1.35-9.07) and respiratory disease (HR 5.00, 95% CI 1.34-18.62); and infection (HR 3.33, 95% CI 1.02-10.92). Mortality as a result of neurological (HR 1.58, 95% CI 1.06-2.35) or genitourinary disease (HR 2.20, 95% CI 1.43-3.37) was also more common in patients with BD during the overall follow-up. Subgroup analyses showed consistent results. The risk of cardiovascular mortality vs. the general population was higher in younger patients (P = 0.006) and the risk of gastrointestinal mortality was increased in women vs. men (P = 0.04). This population-based cohort study revealed that the first year after diagnosis is the highest risk period for excess mortality in people with BD. The mortality burden in BD derives from a wide spectrum of organ involvement and should serve as a warning to clinicians about the systemic nature of the disease.

Comparative analysis of optical coherence tomography angiography (OCTA) results between Behçet's disease patients and a healthy control group.

This study aimed to compare optical coherence tomography angiography (OCTA) findings between patients with Behçet's disease (BD) and individuals with healthy eyes. A cross-sectional study. This cross-sectional study was conducted on patients (67 eyes) with BD who were referred to Feiz Hospital and healthy eyes (43 eyes). All subjects underwent Snellenvisualacuity, a slit-lamp examination, measuring intraocular pressure, conducting a dilated fundus examination, OCTA imaging, and spectral-domain (SD)-OCT imaging. OCTA retinal vascular measurements including optic nerve VD, macular-associated VD( superficial and deep), foveal avascular zone (FAZ) area, FAZ perimeter (PERIM), and vessel density within a 300-μm-wide region of the FAZ (FD) were compared between the groups. A significant difference was evident between the two groups (healthy one group and BD group) in terms of parafoveal and perifoveal total retinal thickness, total pRNFL VD in all quadrants except the inferior sector (P < 0.05), and macular superficial, and deep VD in all regions except temporal and superior perifoveal VD (P < 0.05) following adjustments for age, gender, and signal strength index. When comparing the two groups, ocular Behçet's disease (BD) and non-ocular BD, it was evident that peripapillary vessel density (VD) exhibited a significant decrease in ocular BD eyes in all sectors except for the superior and inferior ones, as compared to non-ocular BD eyes. In addition, the comparison of ocular BD and non-ocular BD showed superficial and deep VDs were lower in ocular BD than non-ocular BD in all regions. According to these findings, peripapillary and macular vessel density is affected in BD. Key Points • The study utilized OCTA to compare retinal features in Behçet's disease (BD) patients and healthy individuals, revealing significant differences in retinal thickness and vessel density. • Ocular BD demonstrated reduced peripapillary vessel density compared to non-ocular BD. • The demonstrated association between ADMA and cIMT in patients with early SSc may suggest a role of NO/ADMA pathway in the initiation of macrovascular injury in SSc.

Does autoimmune disease affect in vitro fertilization results in normo-responder cases?

Aims: This study aimed to assess the effects of autoimmune disorders on pregnancy outcomes in infertile women undergoing in vitro fertilization (IVF) treatment. Methods: This retrospective cohort study was conducted between March 2022 and September 2023, involving 90 infertile women without autoimmune diseases as a control group and 96 infertile women with autoimmune diseases as a case group. The study investigated the impact of autoimmune diseases on IVF treatment outcomes. Single embryo transfer (ET) was performed on the fifth day in all patients. Autoimmune diseases included in this study as Hashimoto's disease, Rheumatoid Arthritis, Sjogren's syndrome, Systemic lupus erythematosus (SLE), Ulcerative Colitis, and Behcet's disease. Results: There was no statistically significant association between the case and control groups regarding live birth rate (p&gt;0.05). Similarly, no statistically significant association was found between the case and control groups concerning the clinical pregnancy rate (p&gt;0.05). Our results revealed that total oocyte, pronuclear (PN), and metaphase II (MII) oocyte rates were statistically significantly higher in healthy infertile women (p&lt;0.05). The number of attempts, anti mullerian (AMH) levels, total gonadotropin dose, and total days were similar between the groups (p&gt;0.05). Conclusion: Autoimmune diseases do not significantly affect pregnancy outcomes in women undergoing IVF. However, several factors, such as total oocyte, PN, and MII oocytes, clinical pregnancy rate (CPR), and live birth rate (LBR) levels, support the association between autoimmune diseases and IVF outcomes.

Рецидивирующий афтозный стоматит: ребамипид – новое направление в лечении и профилактике

Recurrent aphthous stomatitis (RAS) is a widespread disease worldwide, accompanied by the appearance of painful aphthae on the oral mucosa. RAS is a multifactorial disease. Local and systemic factors such as trauma, food sensitivities, nutritional deficiencies, systemic conditions, immunological disorders and genetic polymorphisms are associated with its development. Studies examining the possible connection between the development of RAS and Helicobacter pylori infection are reviewed. The review examined publications on the use of the universal gastro- and enteroprotector rebamipide as a new direction in the treatment and prevention of RAS. Clinical data clearly establish the safety and effectiveness of rebamipide in the treatment of RAS and Behçet's disease. The benefits of rebamipide for RAS include: reduced pain duration, accelerated healing and reduced relapse rates. Aphthous stomatitis (oral mucositis) can be induced by chemotherapy and radiation therapy in oncology practice, with an incidence reaching 100% in patients receiving high-dose chemotherapy and up to 80% of patients receiving radiation therapy. A number of studies in its treatment and prevention have also demonstrated the effectiveness and safety of systemic and local use of rebamipide. The presented review expands the scope of rebamipide, suggesting the possibility of its use in dental and oncological practice for the treatment and prevention of RAS, including Behcet's disease, and oral mucositis.

P1230 Integrated analysis of microbiome and metabolome reveals disease-specific profiles in inflammatory bowel disease and intestinal Behcet’s disease

Abstract Background Gut microbial and metabolic characteristics in intestinal Behcet’s disease (BD), a condition sharing many clinical similarites with ulcerative colitis (UC) and Crohn’s disease (CD), are largely unexplored. This study aimed to investigate the gut microbial and metabolic characteristics, as well as potential biomarkers in intestinal BD, comparing them with UC and CD, along with healthy controls. Methods Patients with UC, CD, and intestinal BD, along with healthy volunteers undergoing diagnostic endoscopies, were enrolled. Colon tissue and stool samples were analyzed using 16S ribosomal RNA sequencing, assessing microbial diversity, taxonomic composition, and functional profiling by phylogenetic investigation of communities by reconstruction of unobserved states (PICRUSt). Plasma metabolomic analysis was performed using gas chromatography and ultra-performance liquid chromatography-time-of-flight mass spectrometry, and quantitative enrichment analysis (QEA) was performed. Results In total, 100 patients (35 UC, 30 CD, and 35 BD) and 41 healthy volunteers were enrolled. While CD exhibited reduced microbial diversity in colon tissue, BD showed no significant decrease. The taxonomic profile of intestinal BD resembled healthy controls more than UC or CD but exhibited specific distinct features. Common changes across all conditions included a decrease in five beneficial bacteria producing short-chain fatty acids (Fusicatenibacter saccharivorans, Coprococcus comes, Blautia obeum, Dorea formicigenerans, and Roseburai ceciola). Additional changes in intestinal BD included a decreased abundance of Subdoligranulum variable and Blautia wexlerae, which were shared features with either UC or CD. Intestinal BD-specific alterations involved decreased abundance of certain bacteria, including Bacteroides fragilis. Metabolomic profiles of intestinal BD by QEA showed similarity to CD and distinction from UC and controls, with pronounced functional changes in energy metabolism and genetic information processing, correlating with microbial functional analysis by PICRUSt. Conclusion This integrative analysis unveiled both common and distinctive profiles in intestinal BD when compared to UC, CD, and controls. The study identified potential biomarkers, contributing to a deeper comprehension of the unique features in these diseases, which could serve as key elements for elucidating their pathogenesis.