Behcet's Disease Research Articles

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.

Hypertension as a prominent manifestation secondary to renal artery lesions in pediatric Behcet’s disease

ObjectiveHypertension caused by vascular Behcet’s disease (BD) is an important prognostic factor of paediatric BD. However, much less is known about its clinical features. The objective of this study was to investigate the clinical characteristics of paediatric vascular BD complicated by hypertension.MethodsA retrospective study was carried out in paediatric BD patients complicated by hypertension treated in the Children’s Hospital Capital Institute of Paediatrics from Jan 2009 to Dec 2022.ResultsOf 65 BD patients, 6 (9.2%) were complicated by hypertension, 5 patients were female, and the median ages of onset and diagnosis were 9.8 years and 11.3 years, respectively. Three patients were found to have cardiac involvement and hypertensive retinopathy secondary to hypertension. Five of the 6 patients with hypertension had right renal artery involvement, and all of them were treated with glucocorticoids and immunosuppressants. Four patients were treated with biological agents. One patient with severe renal artery stenosis underwent unsuccessful vascular interventional therapy. After 3–6 years of follow-up, five patients were found to have renal atrophy, and one patient was at stable condition.ConclusionHypertension in paediatric BD is mainly caused by renal artery involvement. Early recognition and treatment of vascular involvement in BD is important to prevent poor prognosis.

Salivary and Serum Levels of Both Uric Acid and C-Reactive Protein (CRP) Biomarkers in Patients with Behçet's Disease

Behçet's disease (BD) is a systemic vasculitis identified by recurrent oral and genital ulcerations, skin inflammations and uveitis, with unknown etiology. This study had been designed to assess the level of salivary and serum uric acid (UA) and C - reactive protein (CRP) markers in BD patients as activity markers and to explore their relations in disease existence. Fifty BD patients :( 32 males and 18 females) with mean age (35.3 ± 7.6) years and fifty healthy subjects (30 males and 20 females) with mean age (34.8± 9.1) years were involved in this study that extend during the period of (2014-2016). All of patients and healthy subjects were analyzed for UA by (colorimetric spectrophotometric assay that based on oxidation of UA by uricase enzyme to allantoin) and CRP by (qualitative analysis: antigen-antibody interaction Latex agglutination test) in both saliva and serum. Data was analyzed using descriptive statistics, t-test, P (ANOVA) test, Chi-square (2) test, and Pearson’s linear correlation coefficient statistical analyses. Results of this study show that salivary uric acid was significantly elevated in BD patients than in healthy subjects. (P&lt;0.001). Although serum UA was higher in patients than the healthy subjects but statistically not significant. (P=0.57). On the other hand, out of fifty patients there were thirteen patients (26%) significantly expressed CRP positive in their serum and saliva. The results of this study revealed that UA can act as prooxidant (pro inflammatory) marker and CRP as inflammatory marker that could be related to impaired endothelial function in BD.

A Rare Case of Angio-Behcet Revealed by Hemoptysis of Great Abundance

Behçet's disease (BD) is a chronic, relapsing vasculitis of unknown etiology that is characterized by mucocutaneous, ocular, articular, vascular, gastrointestinal, and central nervous system.It mainly affects young subjects, generally 20 to 30 years old with a predominance of men. Thoracic involvement of Behcet's disease is unusual but serious. It is related to the well known vascular tropism of the disease. It may involve the superior vena cava, pulmonary arteries, aorta and subclavian vessels. These vascular attacks can be arterial or venous thrombosis, aneurysms or pseudo-aneurysms .In Behçet's disease, pulmonary artery aneurysms are considered exceptional. these aneurysms manifest themselves by recurrent hemoptysis as is the case with our patient who consulted in pneumolgy department 20 august 1953 at the university hospital Ibn Rochd Casablanca, for a moderate to great abundance hemoptysis revealing an angio-behcet. This is a negative element in patients with BD. it is a diagnostic and therapeutic emergency.The treatment of BD is not well codified but it depends on the severity of the systemic manifestations. Regarding vascular involvement, surgical treatment of an aneurysm with all its risks in patients with Behçet's disease frequently results in aneurysm recurrence. The arterial aneurysms have a poor prognosis, especially pulmonary aneurysms.

Upper gastrointestinal involvement of Behçet's disease in Japan: endoscopic findings and clinical features.

Behçet's disease (BD) can involve any gastrointestinal (GI) tract site. We analyzed the characteristics, risk factors, and treatment responses to upper GI (UGI) involvement in patients with BD. This retrospective cohort study analyzed UGI findings in 101 patients with BD who underwent endoscopy between April 2005 and December 2022 at the University of Tokyo Hospital. The patients were divided into two groups based on the presence or absence of UGI findings. Patient backgrounds, clinical symptoms, colonoscopy (CS) findings, and blood test findings were compared between the groups. In total, 18.8% (19/101) of the patients had UGI lesions. The prevalence rates in the esophagus, stomach, and duodenum were 6.9%, 6.9%, and 8.9%, respectively. Of these 19 patients, BD treatment were intensified in 10 (52.6%) patients after esophagogastroduodenoscopy (EGD), and all showed improvement in symptoms or endoscopic findings. In the multivariate analysis, symptoms (OR: 37.1, P<0.001), CRP>1mg/dL (OR: 11.0, P=0.01), and CS findings (OR: 5.16, P=0.04) were independent predictors of UGI involvement in BD patients. The prediction model for UGI involvement using these three factors was highly accurate, with an AUC of 0.899 on the ROC curve. In the subgroup analysis of intestinal BD, symptoms (OR: 12.8, P=0.01) and ESR>20mm/h (OR: 11.5, P=0.007) were independent predictors. EGD should be conducted in BD patients with high CRP, GI symptoms, and lower GI involvement, which leads to better management of BD in terms of improving symptoms and endoscopic findings.

Trends in health care of patients with vasculitides, including giant cell arteritis, Takayasu arteritis, ANCA-associated vasculitis and Behçet’s disease: cross-sectional data of the German National Database 2007–2021

The aim of this study is to present the current care situation of patients with giant cell arteritis (GCA), Takayasu arteritis (TAK), ANCA-associated vasculitis (AAV) and Behçet’s disease (BD). Trends over the last 15 years will reflect improvements and remaining deficits in the management of vasculitides. Consecutive cross-sectional data from patients with vasculitides from the German National Database (NDB) of the Collaborative Arthritis Centres between 2007 and 2021 were included. Medication, physician- and patient-reported outcomes on disease activity and disease burden, inpatient stays and occupational participation are compared for different vasculitis entities and over time. Employment rates were compared to German population rates. Between 502 and 854 vasculitis patients were annually documented. GCA and AAV were the most common vasculitides. Median disease duration ranged from 2 to 16 years. Over the years, glucocorticoids decreased in proportion and dose, most markedly in GCA and TAK, while biologic therapies increased up to 27%. Physicians rated disease activity as low for the vast majority of patients, while patients-reported moderate outcomes in many dimensions. PROs remained largely unchanged. The proportion of employed patients (< 65 years) increased from 47 to 57%. In recent years, biologics are increasingly used in patients with vasculitides, while glucocorticoids decreased significantly. PRO’s have not improved. Work participation increased but remains lower than that in the German population.

Intestinal Behçet's and suspected intestinal Behçet's disease: a report of four surgical cases.

Intestinal Behçet's disease (BD) is often associated with ulceration that requires surgery, including perforation and abscess formation. However, no consensus has been reached on the optimal extent of resection or treatment strategy. This study reviewed four cases of intestinal or suspected intestinal BD. In Case 1, a 74-year-old woman diagnosed with BD 2years earlier was treated with anti-tumor necrosis factor α antibody (Infliximab) and steroids. She had oral and pubic ulcers. After close investigation of abdominal pain, perforation of the gastrointestinal tract was suspected and surgery was performed. Multiple perforating ulcers and abscesses were found in the distal ileum, and the small intestine was resected. Postoperatively, the patient was treated with an increased steroid dose and symptoms have remained stable. Case 2 involved a 69-year-old woman with oral and pubic ulcers, ocular ulcer, and skin lesions. She experienced sudden onset of abdominal pain during treatment for lymphoma. She showed multiple perforating ulcers throughout the ileum and underwent resection of the small intestine and ileostomy. Upper abdominal pain appeared during postoperative treatment for high-output syndrome. The patient underwent omentoplasty after perforation of the upper gastrointestinal tract was diagnosed. Postoperatively, anti-interleukin-1 beta antibodies (canakinumab) was administered to control the disease. Case 3 involved an 81-year-old, previously healthy woman. She presented to her previous physician with complaints of pubic ulcer, hemorrhage and abdominal pain. Colonoscopy showed multiple ulcers throughout the entire colon. Steroid therapy was started, but bleeding proved difficult to control and total proctocolectomy was performed. Histopathology revealed multiple perforating ulcers and BD was diagnosed. Postoperatively, the patient remains under steroid control. Case 4 involved a 43-year-old man with abdominal pain who showed abscess formation in the ileocecal region. After excision of the ileocecal area, multiple ulcers were diagnosed. Two years later, abdominal pain recurred and free air was found in the abdomen on close imaging. Emergency anastomotic resection was performed due to ulceration and perforation of the anastomosis. Intestinal BD may flare up after surgical treatment and require multiple surgeries. Introducing pharmacotherapy as soon as possible after surgical treatment is important to control the disease.

Does Behcet’s disease affect the quality of sleep?

This research aimed to evaluate the sleep quality (QoS) in individuals with Behçet's disease (BD) compared to a control group of healthy individuals. Additionally, it sought to investigate any possible correlation between BD activity and sleep quality. In this case-control study, individuals with BD and healthy controls, matched for age and gender, were enrolled. The study collected demographic information, body mass indexes (BMI), and disease durations. The BD Behcet disease current activity form (BDCAF) was employed to gauge the disease activity of BD, while the Pittsburgh Sleep Quality Index (PSQI) was utilized to evaluate the QoS. PSQI scores were compared between the patients and controls and the possible correlation between PSQI and BDCAF scores were investigated. Besides, demographic features, disease characteristics, total score and sub parameters of disease activity were compared between BD cases with and without poor QoS. Among 56 BD patients and 56 controls, there were no significant differences in age, gender distribution, or BMI. BD cases demonstrated significantly higher QoS scores than controls (PSQI score: 6.5±4.84 in cases, 3.7±1.48 in controls, p=0.022). While no correlation was observed between PSQI, demographic characteristics, and disease duration, a notable positive correlation emerged between PSQI and BDCAF total score (p=0.037, r=0.458). No statistical difference was determined between demographic data, disease duration, and BDCAF sub parameters between patients with poor (24 patients, 42%) and without poor QoS (32 patients, 58%); however, the total score of BDCAF was significantly higher than those of BD with low QoS (p= 0.021). Our study data suggest that impaired sleep quality in BD cases may be linked to overall disease activity, irrespective of the specific form of involvement. Consideration of QoS in the management of BD will contribute to a holistic approach to the disease.