Behçet's Disease Current Activity Form Research Articles

Expression Levels and Clinical Values of miR-195, miR-424, miR-10b, miR-103a-3p, and miR-542-3p in Vasculo-Behçet's Disease.

MicroRNAs (miRNAs) are involved in a range of pathological and biological processes. Vascular involvement is an important complication associated with morbidity and mortality in Behçet's disease (BD). In this study, we aimed to evaluate the expression levels of miR-195, miR-424, miR-10b, miR-103a-3p, and miR-542-3p in Turkish patients with BD, and their possible association with vascular involvement and clinical activity. This cross-sectional study included 61 BD patients and 25 age- and sex-matched healthy individuals. The patients were categorised into two groups based on the presence or absence of vascular involvement. Demographic data, disease duration, disease activity, and medical treatments were recorded. Disease activity was evaluated using the Behçet's Disease Current Activity Form (BDCAF) and the Behçet's Syndrome Activity Scale (BSAS). The expression levels of miRNAs were measured using real-time quantitative polymerase chain reaction (RT-qPCR). The comparison of the clinical features of BD patients with and without vascular involvement revealed no significant difference. However, the expression levels of miR-195, miR-424, miR-10b, miR-103a-3p, and miR-542-3p were significantly higher in BD patients than in healthy controls (p<0.001, p<0.001, p=0.010, p<0.01, p=0.039, respectively). Moreover, the expression level of miR-195 was significantly higher in vasculo-Behçet patients than in the other groups (p=0.0318). However, no significant association was found between the expression levels of miR-195 and clinical activity. Our study results indicated elevated serum levels of miR-195 in BD patients, which may be associated with vascular involvement. Therefore, miR-195 could potentially serve as a biomarker for the diagnosis and monitoring of vasculo-Behçet's disease.

An Exploration of Some Predictors of Quality of Life-Related to the Innate Immune System, Inflammation, and Disease Activity in Patients with Behcet's Syndrome: An Analytical Cross-sectional Study.

Behçet's disease (BD) has a growing prevalence in Silk Road countries. The aim of our cross-sectional study was to explore the clinical and molecular predictors of quality of life in BD patients. One hundred and fifty consecutive Iranian BD patients with an age range between 20-50 years were included. The Leeds Behçet's disease quality of life (BDQoL) in Persian form was fulfilled to evaluate the quality of life. Anthropometric measurements were carried out using the calibrated scales. Iranian Behcet's Disease Dynamic Activity Measure (IBDDAM), Behcet's disease current activity form (BDCAF), and Total Inflammatory Activity Index (TIAI) were used to assess BD activity. mRNA expression of toll-like receptors 2 and 4 (TLR2 and TLR4) and tumor-necrosis-factor-alpha (TNF-α) levels in serum were measured by real-time polymerase chain reaction (PCR) and ELISA, respectively. Multiple linear backward regression at P = 0.1 was used to study the potential predictors of quality of life. TLR2 and BDCAF were shown to be the most important predictors of quality of life in BD patients by 22%. There were positive associations between them (β = 0.326, p = 0.013 for BDCAF; β = 0.366, p = 0.006 for TLR2) and BDQoL value. Higher TLR2 expression as a key protein in recognizing pathogens by innate immunity and BDCAF value as a comprehensive BD assessing scale contribute to poor quality of life among BD patients. Emphasizing therapeutically, approaches associated with lower TLR2 expression and BDCAF value can be considered in future studies.

Association of high disease activity and serum IL-6 levels with the incidence of inflammatory major organ events in Behçet disease: a prospective registry study.

Little is known about the relationship between the disease activity of Behçet disease (BD) and the incidence of inflammatory major organ events. In this prospective registry study, we investigated the association between the Behçet Disease Current Activity Form (BDCAF) and incidence of inflammatory major organ events, defined as the inflammation of the ocular, central nervous, intestinal, and vascular systems in BD. We enrolled participants from Japanese multicenter prospective cohorts. The BDCAF was evaluated annually. BD-related symptoms, including inflammatory major organ events, were monitored. The association between BDCAF and inflammatory major organ events was analyzed by time-to-event analysis. An unsupervised clustering of the participants' BDCAF, therapeutic agents, and multiple serum cytokines was also performed to examine their association with inflammatory major organ events. A total of 260 patients were included. The patients had a median BDCAF score of 2 [Interquartile range, 1-3] at the enrolment and remained disease active at 1- and 2-year follow-ups, indicating residual disease activity in BD. Patients with a BDCAF score of 0 had a longer inflammatory major organ event-free survival at 52 weeks than those with a score of 1 or higher (p=2.2 x 10-4). Clustering analysis revealed that patients who did not achieve remission despite treatment with tumor necrosis factor inhibitors had high serum inflammatory cytokine levels and incidences of inflammatory major organ events. Among the elevated cytokines, IL-6 was associated with inflammatory major organ events. This study suggests that treatment strategies targeting overall disease activity and monitoring residual serum IL-6 may help prevent inflammatory major organ events in BD.

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.

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.

Assessment of neurocognitive function in patients with Behçet's disease.

There is limited knowledge on cognitive performance in Behçet's disease (BD), the majority of which come from patients with neuro-Behçet's disease. However, the influence of BD on cognitive function in patients without neurological involvement is still not well understood.The aim of the study was to determine the frequency of cognitive involvement in BD patients without evident neuropsychiatric symptoms and to identify associated clinical variables in those patients. Forty BD patients who fulfilled the diagnostic International Criteria for Behçet 's Disease (ICBD) without obvious neuropsychiatric manifestations were studied and compared with forty healthy controls matched for age, sex, and education. A comprehensive medical history, rheumatological, neurological, psychiatric, and psychometric assessment were applied for all patients. Behçet's disease Current Activity Form (BDCAF) was used to assess disease activity. For patients as well as controls, validated Arabic versions of the Wechsler Adult Intelligence Scale-Revised and Wechsler Memory Scale-Revised were used for assessment of cognitive function. Anxiety and depression were additionally assessed for both groups using the anxiety and depression subdivisions of the Arabic Version of Symptom Checklist 90 Revised. Cognitive impairment was identified in 37.5% of BD patients compared to none of the controls. Memory represents the cognitive domain most frequently affected. Cognitive involvement was significantly associated with current corticosteroid use and depression as measured by SCL-90-R. On the other hand, neither the activity of the disease nor the level of anxiety was associated with cognitive involvement. Cognitive dysfunction is reported in BD patients distinctly and independently of clinically overt neurologic involvement. Prevalence of cognitive impairment in patients with BD is strikingly high at 37.5%, whereas the control group exhibited no such signs. Psychological assessment should be performed for every BD patient to reveal any cognitive involvement. It is highly recommended to encourage psychological intervention to prevent any further deterioration, especially in patients who are experiencing depression or currently using corticosteroids.

AB1494 SEXUAL DYSFUNCTION IN BEHÇET’S DISEASE: IS IT AN OVERLOOKED PROBLEM, ESPECIALLY IN MALE BEHÇET’S PATIENTS?

BackgroundBehçet’s disease (BD) is a chronic systemic inflammatory disease characterized by oral and urogenital ulcers, ocular, musculoskeletal, vascular, and neurological involvement. Recent evidence suggests that sexual dysfunction (SD) is prevalent in BD patients.ObjectivesWe aimed to determine the frequency of SD in BD and compare the rates of SD in Behçet’s patients with neurological involvement (NBD), Behçet’s patients without neurological involvement (BDWONI) and healthy controls (HC).MethodsAge matched, sexually active 59 BD (17 NBD, 42 BDWONI) patients and 36 HC were recruited. All participants were questioned with Short Form-36, Beck Depression Inventory (BDI), Golombok-Rust Inventory of Sexual Satisfaction (GRISS), Female Sexual Function Index (FSFI), International Index of Erectile Function (IIEF-5), in addition, disease activity has been assessed by Behçet Disease Current Activity Form (BDCAF) in all BD patients.ResultsThe rate of SD was higher in BD patients (44.2%) compared to HC (14.2%) (p= 0.003)(Figure 1).Male BD patients had lower sexual function scores compared to male HC. Three parameters of GRISS (premature ejaculation, noncommunication, infrequency) were observed more frequently in male BD patients compared with male HC (6.5 vs 4.7, 3.6 vs 2.0, 3.9 vs 2.4, p&lt; 0.05 for all). The SD rates were comparable between female BD patients and HC. SD rates were similar in BDWONI (44.2%) and NBD (42.8%) groups (p&gt; 0.05). BDCAF score was negatively correlated with IIEF scores of male BD patients (r=-0.437) and positively correlated with GRISS scores of female BD patients (r=0.748) (p&lt; 0.05). There was no relation between SD and depression in BD patients (p= 0.284).ConclusionThe frequency of SD is increased in BD compared to HC and affects males more frequently than females. Premature ejaculation, infrequency and noncommunication are common sexual problems in male BD patients. In conclusion, BD has a negative effect on sexuality and BD patients should be evaluated in terms of SD during follow up.

Damage accrual in Behçet disease: Behçet's Syndrome Overall Damage Index (BODI) versus Behçet's Disease Damage Index (BDI).

To assess the validity of Behçet's Syndrome Overall Damage Index (BODI) and Behçet's Disease Damage Index (BDI) as tools for the detection of damage accrual in Behçet's patients compared to Vasculitis Damage Index (VDI). Also, to evaluate the correlation and the interclass correlation among the 3 indices to find out their consistency. A prospective cohort study was carried out on 102 adult Behçet's disease (BD) patients who were diagnosed according to the International Study Group criteria for BD. Disease severity and organ damage were assessed for each patient by VDI, BDI and BODI at baseline and 1-year follow-up visits. Damage accrual for each index was defined when there was an increase of at least 1 point (∆ ≥ 1) among the baseline and the follow-up visits. Correlations among the 3 indices were significant, with (r = 0.835, P < 0.001) between VDI and BODI, (r = 0.835, P < 0.001) between VDI and BDI, and (r = 0.844, P < 0.001) between BODI and BDI scores. A highly significant positive correlation existed between the 3 indices and age and disease duration. In contrast, the correlation with the BD Current Activity Form was non-significant, which indicates good discriminative validity of the 3 indices. Neuropsychiatric and ocular systems showed a strong interclass correlation among the 3 indices. Regarding detecting damage accrual, BDI was more sensitive than BODI and showed more agreement with VDI. BD damage indices, VDI, BODI and BDI, had good convergent and discriminative validity for the assessment of BD damage. BDI had more sensitivity than BODI to the detection of damage accrual.

Metabolic Syndrome in Behçets Disease Patients: Keep an Eye on the Eye.

Metabolic syndrome (MetS) is characterized by insulin resistance, high blood pressure/ sugar, dyslipidemia, and obesity. Whether MetS and its components affect the development of Behçet's Disease (BD) remains unclear. The aim was to determine the frequency of MetS among BD patients and to study its relationship with disease characteristics. The study included 1028 adult BD patients recruited from 18 specialized rheumatology centers. 51 healthy matched control were considered. Behçet Disease Current Activity Form (BDCAF) and the BD damage index (BDI) were estimated. Adult Treatment Panel-III criteria were used to define MetS. The mean age of patients was 36.8 ± 10.1 years, M:F 2.7:1 and disease duration 7.01 ± 5.2 years. Their mean BDCAF was 5.1 ± 4.6 and BDI 5.5 ± 2.8. MetS was present in 22.8% of patients and in 5.9% of control (3.9 fold higher-risk). Patients with MetS had a significantly increased age at onset (31.8 ± 9.2 vs. 29 ± 8.5 years) and higher frequency of genital ulcers (96.2% vs. 79.7%), skin involvement (73.1% vs. 50.4%), arthritis (48.3% vs. 29.1%) (p<0.0001) and CNS manifestations (18.8% vs. 13%) (p=0.042) compared to those without it. Eye involvement was significantly increased in those with MetS (82.1% vs. 74.2%) (p=0.003) with increased frequency of posterior uveitis (67.1% vs. 43.5%), retinal vessel occlusion (35.9% vs. 21.3%), retinal vasculitis (41.9% vs. 26.4%) (p<0.0001) and vitritis (37.2% vs. 24%) (p=0.001). BDCAF was significantly lower (3.9 ± 4.3 vs. 5.6 ± 4.6) and BDI higher (7.4 ± 2.7vs5 ± 2.6) (p<0.0001). BD patients with MetS are predisposed to mucocutaneous, musculoskeletal, neuropsychiatric and ocular manifestations with consequently increased damage. The involvement of the deeper structures of the eye should alarm rheumatologists to keep in mind that all patients should have an eye examination, especially those with MetS.

THE QUALITY OF LIFE OF PATIENTS WITH BEHÇET’S DISEASE AND COMPLEMENTARY AND INTEGRATIVE HEALTH APPROACHES USE TO COPE WITH SYMPTOMS

Aim: This study aims to determine the effect of common symptoms on Quality of Life (QoL) in patients with Behcet Disease (BD) and complementary and integrative health approaches used by patients to cope with these symptoms. Methods: The population of this descriptive study consisted of all patients with BD (n = 330) who are registered with the Behçet’s Disease and Familial Mediterranean Fever Patients Association. The data were collected by the researcher and the president of the association, via an online form between 31.08.2020-06.12.2020. The sample consisted of all patients with BD who met the inclusion criteria (n=247). The data were collected using the Patient Information Form, BD Current Activity Form (BDCAF) and Behçet's Disease Quality of Life Scale (BDQoL). Findings: Joint involvement, rectal bleeding, eye involvement, number of attacks, and disease activity are determinants of 42.5% in affecting QoL (R2 = 0.425, p &amp;lt;0.001). 37.5% of patients with BD have been using CIHA for the last 1 year. They use black mulberry molasses (32%), coriander (9.3%), and thyme juice mouthwash (4%) for mouth sores, wet cupping (24%), and St. John’s wort oil (19%) for genital wounds among CIHA methods. Conclusion: The decisive factor in the use of CIHA in patients with BD was to have genital wounds or loss of sensation in the arm. Therefore, the results of this study are important in terms of leading to further experimental studies to determine the CIHA to be used for symptoms in patients with BD.

Protein Z (rs3024735; G79A and rs3024719; G-103A) gene polymorphisms in Behçet’s disease patients

Aim of the workTo investigate protein Z (PZ) gene polymorphic variations; PZ G79A (rs3024735) and PZ G-103A (rs3024719) in Behçet's disease (BD) patients and their possible relation with disease manifestations, activity and damage. Patients and methodsThis study included 100 BD patients and 100 controls. BD current activity form (BDCAF) and the BD damage index (BDI) were assessed. Genomic DNA analysis of PZ G79A (rs3024735) and PZ G-103A (rs3024719) single nucleotide polymorphisms, was assessed by Real-time PCR-TaqMan SNP genotyping assay. ResultsThe mean age of patients was 33.4 ± 6.8 years and median disease duration was 9 years. AA and GA genotypes of PZ G79A polymorphism were significantly higher in patients than controls (p = 0.003 and p = 0.004 respectively). The frequency of A allele was significantly higher in patients than controls (p < 0.001). There was no difference between patients and controls regarding AA and GA genotypes of PZ G-103A polymorphism (p = 0.5 and p = 0.2 respectively). There was a significant association between AA and GA genotypes of PZ G79A polymorphism with retinal vascular occlusion (RVO) (p < 0.001) and deep vein thrombosis (DVT) (p = 0.003), neutrophil–lymphocyte ratio (p < 0.001) and BDI (p < 0.001). There was no association between PZ G79A genotypes and BDCAF (p = 0.8). A allele of PZ G79A polymorphism was associated with increased BD susceptibility (p < 0.001), RVO (p = 0.001) and DVT (p = 0.01). ConclusionPZ G79A (rs3024735) polymorphism A allele was associated with increased susceptibility to BD with increased risk of developing RVO, DVT and damage, while AA and GA genotypes of PZ G-103A polymorphism were not significantly increased in BD patients.

Characterisation of circulating endothelial microparticles in Behçet's disease: new markers of chronic endothelial damage?

Endothelial cell-derived microparticles (EMPs) are directly indicative of endothelial cell activation or apoptosis, and may also reflect endothelial inflammation, increased coagulation, and vascular tone. The aim of this study is to investigate whether EMPs would be able to evaluate system involvement and be a new indicators of disease activity in Behçet's disease (BD). Thirty-nine consecutive BD patients (who fulfilled the modified 1990 International Study Group on Behçet's disease or the 2006 International Criteria for Behçet's Disease) and 30 age- and sex-matched healthy controls were enrolled. The plasma levels of EMPs were measured by flow cytometry utilising specific labels for endothelial MPs (CD31+ and CD42b-). The levels of circulating EMPs (CD31+ and CD42b-) were significantly elevated in the case group compared with the healthy control group (p =0.000). Moreover, BD patients plasma EMPs were positively correlated with BD current activity form (r=0.802, p =0.000). Vascular and gastrointestinal involvement in BD patients were significantly increased (p=0.004 and p=0.011, respectively) with respect to patients without vascular and gastrointestinal EMPs. Levels of circulating EMPs are elevated in BD patients and correlate with the disease activity; the elevated EMPs may be a potential indicator to predict disease activity of BD. The plasma level of EMPs was increased, which indicated the increased risk of vascular and digestive tract involvement in BD.

Effects of anti-SARS-CoV-2 vaccination on safety and disease exacerbation in patients with Behçet syndrome in a monocentric cohort.

AimVaccination represents a cornerstone in mastering the coronavirus disease 2019 (COVID‐19) pandemic. There is a paucity of data regarding the safety of COVID‐19 vaccines in patients with rheumatic diseases such as Behçet syndrome (BS). The present study aimed to investigate the side‐effects and post‐vaccine disease exacerbation rates of COVID‐19 vaccines in a BS cohort.MethodsWe retrospectively evaluated 450 BS patients followed in our clinic who met the criteria of the International Study Group. COVID‐19 vaccination status, type of vaccine received (Pfizer‐BioNTech vs CoronaVac), post‐vaccine side‐effects and exacerbations were evaluated by interviewing patients over the phone or face to face. Behçet's Disease Current Activity Form (BDCAF) scores were calculated for BS symptoms before and after vaccination.ResultsIn all, 287 patients received at least one dose of the COVID‐19 vaccine. Of the total number of COVID‐19 vaccines (n = 639), 379 (59%) were Pfizer‐BioNTech vaccines and 257 (41%) were CoronaVac vaccines. The number of side‐effects after first, second, third and fourth vaccine doses were 151 (52.6%), 135 (49.4%), 29 (42.6%), and 3 (30%), respectively. BS exacerbation after first, second, third, and fourth vaccine doses were 151 (52.6%), 135 (49.4%), 16 (23.5%), and 3 (30%), respectively. Injection site pain/swelling was the most common side‐effect at all vaccine doses followed by fatigue and arthralgia.ConclusionCOVID‐19 vaccines are well tolerated in patients with BS, and more side‐effects develop after mRNA vaccines. Regardless of the vaccine type, exacerbations after the COVID‐19 vaccine are common, predominantly mucocutaneous and articular involvement, and exacerbations in the form of other organ involvement are rare.

Association of long non-coding RNAs NEAT1, and MALAT1 expression and pathogenesis of Behçet's disease among Egyptian patients

Behçet's disease (BD) is a chronic inflammatory disease. Immunological defects have been shown to play a significant role in the progression of BD. The serum levels of two long non-coding RNAs (lncRNAs), NEAT1 and MALAT1, were examined in patients with BD to identify their role in the disease pathogenesis. Both lncRNAs were mentioned as essential regulators of innate immune responses and have a crucial role in inflammatory diseases. Fifty patients with BD and a similar number of control individuals were involved in our study. At enrollment, data was collected from patients and controls, and the disease severity in active cases was determined using the Behçet's Disease Current Activity Form (BDCAF). Levels of the two studied biomarkers in the serum, NEAT1 and MALAT1, were investigated by quantitative RT-PCR (qRT-PCR). NEAT1 levels were significantly turned down in BD patients (fold changes = 0.77, p = 0.0001) and correlated negatively with the BDCAF (r = −0.41; p = 0.003). On the other hand, the MALAT1 levels were significantly up-regulated in BD patients (fold changes = 2.65, p = 0.003). Serum levels of NEAT1 were significantly decreased in patients with active states than in stationary cases (0.387 versus 1.99, respectively; p = 0.01) and compared with controls (p = 0.001). Also, NEAT1 levels were significantly increased in patients with stationary states compared to controls (p = 0.03). There was a positive association between NEAT1 and MALAT1 levels among BD patients (r = 0.29, p = 0.04). Our findings demonstrate a possible role of NEAT1 and MALAT1 in the pathogenesis of BD.

Immunomodulatory and clinical responses to zinc gluconate supplementation in patients with Behçet's disease: A double-blind, randomized placebo-controlled clinical trial

Toll-like receptor (TLR) 2 and 4 are involved in the pathogenesis of Behçet's disease (BD). The current study aimed to investigate the effect of zinc supplementation on TLR-2/4 expression and the clinical manifestations of BD. In this double-blind placebo-controlled randomized clinical trial, 50 BD patients were randomly allocated into either zinc gluconate (30mg/day) or placebo groups for 12 weeks. Before and after the intervention, the surface and mRNA expression level of TLR-2 and TLR-4 in the leukocytes, serum level of zinc and tumor necrosis factor-α (TNF-α), quality of life, anthropometric measures, and blood pressure of patients were collected. BD activity was studied using the nonocular Iranian Behçet's disease dynamic activity measure (IBDDAM), Behçet's disease current activity form (BDCAF), and total inflammatory activity index (TIAI) at the pre-and post-intervention phases. The effect sizes were compared between two groups using analysis of covariance. There were significant decrease in TLR-2 mRNA (P=0.038) and protein expression (P=0.034) and nonocular IBDDAM score (P=0.046) in the zinc group compared to placebo at the endpoint. The serum level of zinc was increased in the zinc group (P<0.001). Zinc supplementation significantly decreased the TLR-4 surface (P=0.012) and mRNA expression (P=0.028) within the group. However, this decrease was not significant compared to the placebo group. There was no significant difference between the two groups regarding the serum level of TNF-α, BDCAF, TIAI, quality of life, anthropometric measures, and blood pressure (P>0.05). The present study revealed that zinc supplementation significantly improved nonocular IBDDAM score and TLR-2 expression in BD patients. NCT05098678.

Anti-tubulin-alpha-1c antibody as a marker of value in Behçet syndrome

BackgroundBehçet’s syndrome (BS) is a multi-systemic vasculitis characterized by recurrent oral ulcers, genital ulcers, ocular lesions, and other systemic manifestations. As there is no laboratory diagnostics of BS, the diagnosis is mainly clinical.ObjectiveTo investigate the utility of the autoantibody against tubulin-α-1c in diagnosis of BS and its clinical significance.MethodsSixty BS patients and sixty healthy controls were enrolled in this study. We assessed all patients by Behçet disease current activity form (BDCAF), routine laboratory investigations, and immunological markers (ANA, anti-DNA, ANCA). Anti-endothelial cell antibodies (AECA) and anti-tubulin-alpha-1c antibodies were performed for all participants.ResultsRegarding duration of illness, Birmingham Vasculitis Activity Score (BVAS), and BDCAF, the mean value was 4.77 ± 4.239, 19.80 ± 10.020, and 9.52 ± 5.476, respectively. On comparing laboratory investigations, there was only significant increase in anti-tubulin-alpha-1c antibody in BS patients compared to healthy controls. Regarding AECA, there was no any significant correlation except with CRP. Anti-tubulin-alpha-1c detected significant direct correlation with the presence of posterior uveitis, panuveitis, and venous thrombosis as well as BVAS, C4, and protein/creatinine ratio. Regarding diagnostic performance of both AECA and anti-tubulin-alpha-1c, the cutoff value of AECA for diagnosis was 27.250, with sensitivity and specificity of 93.3% and 96.7%, respectively. The cutoff value of the anti-tubulin-alpha-1c for diagnosis was 22.300, with sensitivity and specificity of 100% and 96.7% respectively.ConclusionAnti-tubulin-α-1c antibodies are of diagnostic value in BS and are indicative of activity with 100% sensitivity and 96.7% specificity.Key Points• There is lack of specific laboratory, radiological, or histological diagnostics for Behcet syndrome.• We aimed to evaluate the significance of tubulin-α-1c autoantibody in diagnosis of Behcet syndrome.• There is elevation of tubulin-α-1c autoantibody with sensitivity and specificity of 100% and 96.7%, respectively.

Computed tomography pulmonary angiography (CTPA) in Behçet's disease patients: a remarkable gender gap and time to refine the treatment strategy.

The aim of the work was to delineate the computed tomography pulmonary angiography (CTPA) findings in Behçet's disease (BD) patients with and without chest manifestations. The study included 122 BD adults recruited from 5 Teaching University Hospitals in Egypt of those who agreed to perform a CTPA. The Arabic version of BD current activity form (BDCAF) and BD damage index (BDI) were assessed. Detailed pulmonary manifestations, examination, plain radiology chest, and CTPA findings were recorded. The mean age of patients was 36.9 ± 11.3years, male:female was 1.8:1, disease duration 9.6 ± 8.2years, and age at onset 28.3 ± 8.6years. Their mean BDCAF was 4.4 ± 2.2 and BDI 3.4 ± 1.8. There were chest manifestations in 51 (41.8%) and plain chest x-ray findings in 13 (10.7%) and CTPA findings in 31 (25.4%) in the form of pulmonary thromboembolism in 15 (12.3%), pulmonary aneurysms in 7 (5.7%), pneumonia in 5 (4.1%), interstitial lung disease in 4 (3.3%) and pleural effusion in 3 (2.5%). Patients with chest manifestations had significantly higher frequency of cardiac manifestations (15.7%) compared to those without (2.8%; p = 0.023); chest x-ray findings tended to be higher (17.6% vs 5.6%; p = 0.05) while CTPA findings were significantly detected (51% vs 7%; p < 0.0001). Higher frequency of CTPA findings were in females (p < 0.0001). Yet the rate of serious pulmonary embolisms, aneurysms, and thrombosis was exclusive in males. Meticulous investigation of the chest manifestations is warranted in BD patients to undermine the actual magnitude of pulmonary impact. CTPA provides a realistic estimate of the extent of involvement even in asymptomatic cases. Key Points • Meticulous chest assessment is warranted in Behçet's disease patients to undermine the actual magnitude of pulmonary impact • CTPA provides a realistic estimate of the extent of involvement even in asymptomatic cases.

Clinical Characteristics of Behçet Disease in an Egyptian Cohort

Abstract Aim of the work To study the clinical characteristics of Behcet’s disease (BD) in Egyptian patients. Patients and methods 150 BD patients were recruited from the Rheumatology and Internal medicine Department, Ain Shams University Hospitals. Demographic and clinical data were collected. Disease activity was assessed using Behcet Disease Current Activity Form (BDCAF). Results 150 BD patients were included in this study. Their ages were 34.05 ± 9.28 years (14 – 66 years) and with disease duration range (1–20 years). There were 116 males (77.3%)and 34 females (22.7%), with male to female ratio of 3.4:1. The BDCAF was 4.44 ± 1.97 (2–10). The frequency of the clinical manifestations was oral ulcers in 86.7%, followed by genital ulcers in 82.0%, ocular involvement in 68.7%, vascular affection in 58.7%, articular manifestations in 32.7%, pathergy test was positive in 28.0%, skin manifestations in 26.0% (pustular lesions, folicilitis,erythema nodosum and pyoderma gangrenosum), neurological in 25.3% in the form of (CNS infarction in 12.7%,vasculitis in 6.7%, Cerebral sinus thrombosis in 4.0% and Seizures in 2.0%) and gastrointestinal in 7.3% in the form of (nausea, vomiting and abdominal pain). Conclusion In Egypt, there is a male predominance in BD patients as other areas in worldwide countries. The clinical characteristics are comparable to other studies. However, in Egypt the gastrointestinal manifestations showed the lowest recorded frequency

Cardiovascular Manifestations of Behçet Disease among Egyptian Patients

Abstract Background Behcet’s disease is an auto immune disease with multisystem affection, the most common manifestations are in the form of oral or genital ulcers, eye affection, recurrent thrombosis . Behcet’s disease is a chronic disease with many sequelae, sometimes fatal, the treatment is by immunosuppressive therapy which also had its implications on the long term, unfortunately it has no sure diagnostic tests and relies mainly on clinical criteria, there are few scores to assess behcet’s disease activity (Behcet's Disease Current Activity Form) BDCAF score is a reliable follow up tool with wide accreditation. BD can virtually involve any organ system, cardiac involvement is variable and sought to occur in the range of 7-46% of patients and may cause myocardial infarction, pericarditis, valve problems, aneurysms or congestive heart failure. Different types of vessels, predominantly veins, can be affected in BD. The frequency of vascular lesions in BD, such as superficial and deep venous thromboses, arterial aneurysms and occlusions, ranges between 7-29%. Aims To assess cardiovascular manifestations for patient diagnosed as Behçet disease (BD) and its correlation to disease activity. Methods This was a cross sectional study which was done on 40 Behcet’s disease patients within the year 2019, it was done for the assessment of cardiovascular manifestations for patient diagnosed as Behçet disease (BD) and its correlation with BDCAF score (Behcet’s disease current activity form). The study group was subjected to full history, clinical examination including activity which was assessed according to BDCAF score (BEHCET'S DISEASE CURRENT ACTIVITY FORM), Assessment of any activity regarding the eyes using fundus examination, routine laboratory investigations including Complete blood count (CBC), Erythrocyte sedimentation rate (ESR), Creactive protein (CRP), lipid profile, liver enzymes. Results it was found that there is a positive correlation between BDCAF (behcet's disease current activity form) and cardiovascular affection (especially valvular lesions and vascular venous affection).

Heart Rate Variability as an Indicator of Autonomic Nervous System Disturbance in Behcet's Disease.

BackgroundBehçet’s disease (BD) is a multisystemic vasculiti where cardiac involvement is not common. Previous studies have shown that the incidence of ventricular arrhythmia and sudden cardiac death (SCD) is higher in patients with BD than in the healthy population. Among various possible explanations, autonomic nervous system (ANS) dysfunction has been suggested. Few studies have evaluated ANS function in patients with BD, and the results obtained are controversial.ObjectiveWe aimed to assess cardiac autonomic function by heart rate variability (HRV) in patients with BD; to evaluate circadian changes in HRV; and to study the relationship between HRV parameters and disease parameters.MethodsThirty-six patients with Behçet’s disease (median age 42 years, 28 men) and 36 age- and sex-matched healthy volunteers were included. HRV analyses were performed in the time and frequency domains for the entire 24-hour period and for the daytime and nighttime periods. BD activity was studied with Behcet’s disease current activity form (BDCAF).ResultsPatients with BD had significantly lower values of SDNN compared to controls. PNN50, RMSSD, and HF components mean values were significantly reduced in patients than in controls implying parasympathetic impairment. LF was comparable between the two groups, whereas LF/HF ratio was significantly higher in BD group. The circadian rhythm of HRV was preserved in patient group. There was no significant correlation of CRP or disease duration with HRV indices in Behçet’s subjects. BDCAF score was found to be negatively correlated with LF/HF.ConclusionPatients with BD, despite no cardiovascular involvement, have reduced parasympathetic activity compared with controls. However, circadian rhythms of autonomic function were preserved. As known, there is strong evidence for the role of the ANS in the pathogenesis of ventricular arrhythmias. Thus, being a practical tool, HRV can be an interesting approach for the rhythmic follow-up of BD patients.