Behcet's Syndrome Research Articles

Vascular thrombosis and relapse-associated factors in Behcet's syndrome: a comprehensive analysis of 136 patients

Abstract Background Vascular thrombosis in Behçet syndrome (BS) is frequent and associated with high morbidity and mortality rates. Purpose To investigate BS patients presenting with a thrombotic event (TE) and identify the factors associated with relapse. Methods We retrospectively conducted a single-center, descriptive, and analytic study, from 2000 to 2022, enrolling all BS patients presenting TE, including venous thrombosis (VT) and/or arterial thrombosis (AT). Results TE was observed in 136/531 patients (25%), mostly men (83%), aged 29 ± 9 years (11-50) at BS onset. TE was the initial presentation of BS (32%) or occurred after a median of 4 years [0;25]. It included VT (92.5%), AT (18%), and both (13%). VT mostly consisted of DVT in the lower extremities (60%), vena cava (30%), and cerebral (15%) veins. It involved one (55%), two (27%), and multiple (16%) veins (Figure 1). AT mainly affected the pulmonary (42%), femoral (21%), coronary (17%), cerebral (8%) arteries, and aorta (8%). Arterial aneurysms were common (18%), especially in the pulmonary arteries (11%). Intracardiac thrombosis was also visualized (10%). Noncardiovascular manifestations mainly included aphthous ulcers (96%), pseudofolliculitis (36%), ocular lesions (41%), and arthralgias (22%). Hemostasis parameters were within normal ranges. Patients were treated with colchicine (98%), corticoids (98%), and immunosuppressants (80%). Anticoagulation and anti-platelet aggregation were administered in 90% and 15% of the cases, respectively. Six patients underwent vascular surgery. Post-thrombotic syndrome was noted in 14% of the patients. Relapse was common (25%), mainly occurring in men (81%), of younger age (24 ± 6) at first TE. Relapse consisted of one (55%), two (32%), or multiple episodes (13%). It occurred in the same vascular system initially affected (69%), a different one (12%), and both at the same time (19%). Multivariable analysis revealed TE at onset (OR= 2.99, 95% CI[1.14 ; 7.82], p=0.02), arterial thrombosis (OR= 2.91, 95% CI[1.10 ; 8.40], p=0.04), and arterial aneurysms (OR= 2.58, 95% CI [1.09 ; 8.45], p=0.02) as factors significantly associated with TE relapse (Figure 2). Conclusion Our study highlights several particularities of TE in BS, with TE at onset, arterial thrombosis, and arterial aneurysms emerging as significant relapse factors.

Pulmonary artery thrombosis in Behcet's syndrome: a case series

Abstract Background Behçet's syndrome (BS) is a complex multisystemic vasculitis that affects vessels of all sizes and kinds, with a high tendency towards thrombosis. Arterial disease is rare and manifests primarily in the form of pulmonary artery aneurysms (PAA), but also of pulmonary artery thrombosis (PAT) in 1/3 of the cases. Purpose Our work aims to depict the profile and outcomes of BS patients presenting with PAT. Methods This retrospective, descriptive, and monocentric study was conducted between 2000 and 2022, among 531 adult patients diagnosed with BS (ISG and/or ICBD criteria). All cases of documented PAT were included. Results 16 cases of PAT were recorded (3%). The mean age of the patients was 34 ± 6 years (19-58), and the sex ratio (M/F) was 4.7:1. Cardiovascular risk factors were mainly active smoking (12%) and high blood pressure (6%). PAT was the revealing manifestation of BS (30%), or occurred after a median of 4.5 years following BS onset. Patients were mostly asymptomatic (69%), or presented with dyspnea (35%), hemoptysis (31%), and thoracic pain (25%). On chest CTA, PAT was often bilateral (78%) and proximal (46%). PAA were frequently observed (25%), being consistently bilateral, multiple, proximal and distal at the same time. Deep venous thrombosis was found in the lower extremities (19%), superior vena cava (27%), and inferior vena cava (6%). Cardiac involvement was also commonly associated (40%), mostly consisting of right intracardiac thrombosis (37%). Concomitant extracardiovascular involvements included mucocutaneous (87%), ocular (26%), articular (25%), and digestive (6%) lesions. Inflammatory parameters were raised in most patients (80%). Hemostasis parameters were within normal ranges and screening for inherited and acquired thrombophilia was negative in all cases. Patients were treated with colchicine (97%), glucocorticoids (93%), cyclophosphamide (47%), azathioprine (47%), and a TNF-a-inhibitor (6%). Curative anticoagulation with low-molecular-weight heparin followed by oral antivitamin K was often prescribed (60%). No surgery or endovascular procedure was performed. Relapse and death were recorded in respectively 15% and 26% of the cases. Conclusion In contrast to common thrombotic diseases, PAT in BS is attributed to vasculitis rather than hypercoagulability, resulting in distinct clinical features. The diagnosis should be promptly considered in young men who present with concurrent thrombotic lesions and PAA. Early and appropriate management is imperative to enhance patient outcomes.Left proximal PAT in a BS patient

Nuclear factor kappa B 1 A > G single-nucleotide polymorphism (rs4648068) in Egyptian patients with Behcet’s syndrome, case–control study

BackgroundBehcet's syndrome (BS) is a variable-vessel vasculitis characterized by hyperactive innate immunity. The nuclear factor kappa B (NFKB) pathway is involved in the regulation of inflammatory responses including innate and adaptive immune responses. BS could be associated with NFKB hyperactivation. We aimed to study the association between the NFKB1 A > G (rs4648068) single-nucleotide polymorphism (SNP) and BS in Egyptian patients, in comparison to healthy controls, and to correlate the presence of rs4648068 SNP with the different activity domains of the disease. After ethical committee approval (Faculty of Medicine, Cairo University, Egypt, code MD-228-2022), the International Study Group Criteria for Behçet's Disease (ISG) criteria was used to recruit 60 BS patients, and the activity of the disease was assessed using Behcet’s Disease Current Activity Form (BDCAF) and the Behcet Syndrome Activity Score (BSAS). Another 60 matched controls were recruited. DNA extraction was done followed by PCR amplification to detect the target SNP.ResultsThe GG genotype was significantly higher in BS versus controls (21.7% and 5%, respectively, p value = 0.015). Also, the G allele was significantly higher in BS versus controls (43.3% and 30%, respectively, p value = 0.033). Of the whole activity domains, only arthralgia was found to be significantly correlated with rs4648068 SNP.ConclusionNFKB1 rs4648068 A > G SNP increases the risk of developing BS. Among patients with BS, the GG genotype is protective against developing arthralgia. There is no statistically significant relation between rs4648068 SNP and either other activity domains of BS or the different activity scores of the disease.

Risk of Postoperative Complications After Total Hip and Total Knee Arthroplasty in Behcet Syndrome Patients.

Behcet syndrome (BS), a multisystem autoimmune disorder, has unclear effects on outcomes after total hip arthroplasty (THA) and total knee arthroplasty (TKA). This study assessed the relative risk of perioperative adverse events in patients with BS. This retrospective cohort study used the PearlDiver M157Ortho data set, a large national administrative database. Total hip arthroplasty and TKA patients with BS were identified and matched 1:4 to those without BS based on patient age, sex, Elixhauser Comorbidity Index scores, and procedure performed (THA or TKA). The incidence of 90-day adverse events was determined and compared by multivariate analysis. 5-year survival to revision surgeries was assessed and compared with the log-rank test. After matching, 282 THA/TKA patients with BS were identified and compared with 1127 without BS. On multivariate analysis, patients with BS were at independently greater risk of aggregated any (odds ratio [OR] 2.16, P < 0.0001), serious (OR 1.78, P = 0.0051), and minor (OR 2.39, P < 0.0001) adverse events compared with those without BS. No significant difference was observed in 5-year survival to revision surgery (P = 0.3). Patients with BS undergoing THA or TKA experienced markedly greater 90-day postoperative adverse events. The findings underscore the need for optimized perioperative management for patients with BS undergoing arthroplasty.

The impact of environmental factors on aetiopathogenesis and clinical manifestations of Behçet's syndrome.

Behçet's syndrome (BS) is a rare multisystem vasculitis involving blood vessels of any size. BS aetiology is still unclear to date, and the heterogeneity of clinical expression among ethnics and genders make early diagnosis challenging. However, so far, considerable efforts have been made toward the understanding of BS, leading researchers to agree that the coexistence of some environmental triggers and a genetical susceptibility both underlie BS aetiopathogenesis. In particular, viral agents, oral microbial flora, and mucosal microbiota have been widely explored in this regard, but still no specific microorganism has been definitely linked to the disease aetiology. Likewise, the concept that some environmental factors may play a role in BS clinical presentation has emerged based on the growing evidence that disease severity is usually higher in male patients, and that diet and fatigue may be involved in disease recurrence, especially in mucocutaneous manifestations. Moreover, smoke cessation is acknowledged as a risk factor for oral ulcerations, although the underlying mechanism is still not clear. All those environmental factors play their effects through epigenetic mechanisms. The aim of this review is to discuss the evidence on the role of environmental factors in BS aetiopathogenesis and clinical course.

Frailty assessment in patients with Behçet's syndrome: A cross-sectional monocentric study

AimsEvidence evaluating the association between pre-frailty and frailty, and risk of adverse health outcomes in patients with Behçet's syndrome (BS) is limited in the literature. The aim of this study was to characterize the prevalence of frailty and associated factors in a single-centre cohort of patients with BS. MethodsBased on the International Study Group's criteria, this was a monocentric cross-sectional study of BS patients. The Fried frailty criteria were used to define frailty. The Turkish version of the Behçet's Disease Current Activity Form was used to measure the disease activity of BS. Damage index was assessed with the Behçet's Syndrome Overall Damage Index. ResultsForty-four patients were enrolled. According to Fried frailty criteria, patients were classified as 13.6% frail, 59% pre-frail, and 27.2% robust, respectively. Compared to pre-frail and robust patients, frail patients had higher levels of inflammatory markers at the time of diagnosis. CRP levels at time of diagnosis and at the last visit were higher in the frail group than in the pre-frail and robust groups (p=0.039 and p=0.023, respectively). When active drugs for BS were evaluated, systemic glucocorticoid (50%, p=0.030) and cyclophosphamide (33.3%, p=0.006) treatments were higher in the frail group. ConclusionsFrailty and pre-frailty are commonly detected even in younger patients with BS. Inflammation can be described as potential determinants of frailty status.

Update on Behçet syndrome

Behçet syndrome (BS) is avasculitis of variable vessels with multiple organ manifestations. This article gives an overview of innovations in the last 2 years. A literature search was carried out using the keyword "Behcet" in 2022-2024 in PubMed. The selection of suitable articles was based on the relevance. With respect to the pathophysiology it is now clear that BS occupies an intermediate position between autoinflammatory and autoimmune clinical pictures. It is now classified as MHC-I-opathy, i.e., adisease that has astrong association with HLA classI antigens, which also play aprominent role in the pathogenesis. The diagnostic international criteria for Behcet's disease (ICBD) from 2014 with ascore of 4 points or more that makes the diagnosis of BS probable have become established; however, in countries with alow prevalence of BS, the differential diagnosis of BS from other diseases is difficult and ahigher point limit in the diagnostic score seems to make sense in order to avoid incorrect diagnoses. Clusters or phenotypes of the disease have now been described in various countries in which different symptom complexes frequently occur together; however, the clusters differ between the different countries of origin and depending on the age of the patients. Sonography of the common femoral vein with specific wall thickening in BS patients has been established as an additional tool for the differential diagnosis. Typical characteristics of oral aphthae in BS were also described and the frequency of positivity in the pathergy test could be significantly increased using pneumococcal antigens as the reagent. The treatment recommendations of the EULAR from 2018 still apply; in treatment-refractory cases, tocilizumab, secukinumab, Janus kinase inhibitors (JAKi) and ustekinumab have now also been successfully used. The new EULAR treatment recommendations are expected in 2025.

Clinical characteristics of Behçet's syndrome in Shanghai database: Baseline data of a cross-sectional cohort study.

Behçet's syndrome (BS) is a variant vessel vasculitis that can involve multiple organs, with highly heterogeneous clinical manifestations. This study aims to analyze baseline data of BS patients to enhance the comprehension of its clinical features. This study included 1216 registered cases of BS patients referred to Huadong Hospital affiliated with Fudan University. Each patient was thoroughly assessed and recorded for demographic data, clinical manifestations, gastrointestinal endoscope, imaging, etc. RESULTS: Significant gender differences were observed in clinical manifestations. Pseudofolliculitis (p < .001), uveitis (p = .003), vascular (p < .001), and cardiovascular involvement (p < .001) were significantly more prevalent in male BS patients, while genital ulcers (p = .011) and erythema nodosum (p = .009) were more common among the female. Furthermore, pseudofolliculitis (44.3%, 37.4% vs. 25.0%, p < .001), pathergy test positivity (37.0%, 24.5% vs. 12.6%, p < .001), and uveitis (18.8%, 18.4% vs. 11.2%, p < .001) showed higher incidence rates in the 16-35 years age group. Vascular involvement (11.1%, 18.0% vs. 15.8%, p < .001) notably increased in the 36-50 years age group. Additionally, the ISG diagnostic criteria were more likely to be met in the 16-35 age group (OR: 2.039, 95% CI: 1.581-2.631, p < .001), whereas the ICBD criteria were less likely to be met in the 16-35 age group (OR: 0.266, 95% CI: 0.150-0.474, p < .001). This study provided data on the baseline of clinical features of BS in a single center, BS patients presented significant heterogeneity, showing different manifestations across various genders and age groups. This diversity might contribute to a better understanding of BS clinical features and pave the way for future multi-center studies.

Tocilizumab may not be a good option for vascular involvement due to Behçet's syndrome.

Tocilizumab has been increasingly reported as an alternative therapeutic agent in the management of Behçet's syndrome (BS) and it has been mostly tried in BS patients with neurological and eye involvement. As therapeutic responses to each drug may vary across different types of BS involvement, we aimed to report seven patients with large vessel involvement treated with tocilizumab. We enrolled seven BS patients with vascular involvement who were given tocilizumab at the Behçet's Disease Research Centre in Istanbul University-Cerrahpaşa between 2000 and 2022. Demographic information, BS features, types of vascular involvement, previous and concomitant medications, C-reactive protein (CRP) levels, imaging modality results, and outcomes were documented from the patients' medical records. Within a median of 6 months after the initiation of tocilizumab, 5 patients experienced vascular relapses. These relapses included the emergence of new bilateral pulmonary artery aneurysms, a new pulmonary artery thrombus, parenchymal lung involvement, deep vein thrombosis in the lower extremity, and pseudotumor cerebri in one patient each. CRP levels were normal in 4 of the 5 patients at the time of vascular relapse. One of these 5 patients and another patient with aortitis had an exacerbation of mucocutaneous symptoms. In the last patient, venous ulcers did not respond to tocilizumab and were complicated with infection. Tocilizumab could potentially exacerbate vascular manifestations, similar to what is observed with mucocutaneous lesions in BS patients. Furthermore, CRP levels appear to be ineffective in monitoring these patients.

Sexual dysfunction among female patients with rheumatic diseases.

To demonstrate the burden of sexual dysfunction (SD) among females with rheumatic diseases, we conducted a cross-sectional comparative study in patients with systemic sclerosis (SSc), systemic lupus erythematosus (SLE), and Behçet's syndrome (BS) along with suitable healthy controls (HCs). Age-matched female patients with SSc (n = 50), SLE (n = 49), and BS (n = 54), along with 52 female HCs were included in this study between April and October, 2021. Sociodemographic features were recorded, and psychometric tests, i.e., female sexual function index (FSFI), Beck depression inventory (BDI), body cathexis scale, and marital adjustment test (MAT) were performed. Scale scores were compared, and binary logistic regressionwas usedto identify predictors for SD in the whole group. The total FSFI and body cathexis scores among the patient groups were significantly lower than those of the HCs (p < 0.001). Depression was significantly more frequent in the patient groups. MAT scores did not differ significantly between the study groups. Patients with SSc had the worst scores in each psychometric index, including MAT. Decreased body cathexis score [OR 0.974, 95% CI (0.957-0.991), p = 0.003] and low MAT score [OR 0.937, 95% CI (0.896-0.980), p = 0.005], and being diagnosed with SSc [OR 6.6, 95% CI (1.975-22.498), p = 0.002], SLE [OR 2.7, 95% CI (0.998-7.753), p = 0.050], and BS [OR 2.8, 95% CI (1.100-7.359), p = 0.031],were identifiedas independent predictors for SD. Body cathexis seems to be the most important independent predictor for SD, and the burden of SD appears heavier in patients with SSc, probably due to poor body image satisfaction.

Challenges and opportunities in transitional care process in Behçet's syndrome.

Behçet's syndrome (BS) is a rare chronic multi-systemic inflammatory disorder that usually involves adults between third and fourth decades of life, while pediatric and juvenile onset are relatively rare. BS young patients (YP) often develop a full-blown disease late after onset, requiring careful diagnostic workup and regular follow-up while they grow up. In this regard, the purpose of transitional programs is to ensure continuous high-quality care to YP with chronic conditions, providing them with the skills necessary to become independent and empowered adults able to chronically self-manage their disease. EULAR/PReS released the first set of standards and recommendations for transitional care (TC) of YP with juvenile-onset rheumatic diseases, but the appropriate timing for transition, the tools to evaluate patients' readiness, and indicators of transition plans effectiveness still need to be identified. Although little is known regarding TC in BS, it is easy to assume that BS YP will benefit from developmentally and disease-specifically appropriate transition plans, which may promote continuity of care, improve perceived quality of life and prevent poor disease outcomes. This perspective article discusses the key concepts and the goals of TC, addressing the potential challenges and opportunities of TC for YP with BS in clinical practice.

The Current and Novel Imaging Modalities for Ocular Vasculitis in Behcet's Disease: A Review.

Behcet's syndrome is a multisystem inflammatory disorder characterized by a chronic relapsing course and diverse clinical manifestations, prominently affecting young adults along the ancient Silk Road and beyond. The disease pathogenesis involves complex interactions between genetic predisposition, environmental triggers, and dysregulated immune responses, leading to systemic vasculitis and tissue damage. Ocular involvement, a hallmark of Behcet's Disease (BD), significantly impacts morbidity, with uveitis as a common initial presentation that can progress to severe vision-threatening complications like retinal vasculitis and occlusive disease. This review consolidates current knowledge on ocular manifestations in BD, emphasizing the pivotal role of multimodal imaging in diagnostic evaluation and management. Fundus photography serves as a baseline tool for documenting intraocular lesions and monitoring treatment responses. Fluorescein angiography remains the gold standard for detecting acute inflammatory changes and vascular leakage patterns essential for disease staging and prognostication. Recent advancements in imaging, such as ultra-wide field imaging (UWF), indocyanine green angiography (ICGA), Doppler ultrasonography, Optical Coherence Tomography (OCT), OCT angiography (OCTA), adaptive optics (AO), and retinal function imaging (RFI), provide unprecedented insights into microvascular dynamics, structural changes, and functional impairments associated with ocular BD. Integration of these advanced imaging modalities enhances early detection of subclinical disease, facilitates precise localization of inflammatory lesions, guides therapeutic interventions, and monitors treatment efficacy. OCT and OCTA, in particular, offer non-invasive, high-resolution assessments of macular edema, vascular perfusion abnormalities, and choroidal thickness alterations critical for optimizing patient care. In conclusion, multimodal imaging represents the cornerstone in the comprehensive management of ocular manifestations in Behcet's Disease, offering clinicians invaluable tools for accurate diagnosis, treatment planning, and long-term monitoring of disease progression and treatment outcomes.

The distribution of biomarkers for Behcet syndrome and their clinical relevance in real-world studies

Objective: To explore the distribution of different biomarkers for Behcet's syndrome (BS) and their correlation with distinct clinical phenotypes of BS patients in real-world studies. Methods: This study was a retrospective cross-sectional study. A total of 483 patients diagnosed with BS in the Department of Rheumatology and Immunology, Peking University People's Hospital from 2019 to 2022 were enrolled. The baseline information and clinical features of the patients were recorded at their first diagnosis and tested the level of HLA-B51, several auto-antibodies, antistreptolysin-O(ASO), immune globulin, complement in blood serum and interleukin-6 (IL-6). Logistic regression was used to analyze the correlation of biomarkers and phenotypes. Results: Among BS patients, the number of positive cases for HLA-B51, anti-endothelial cell antibody (AECA), antinuclear antibodies (ANA) and ASO was 129, 115, 79 and 54, respectively. The positive rate of other biomarkers was less than 5.0%. About 12.6% of patients with BS had an increased level of IgA (n=61), and 10.8% of patients had an increased level of IgG (n=52). About 41.0% of patients had increased levels of IL-6 (n=198), and 6.4% of patients had decreased levels of IgM (n=31). About 11.2% of patients had decreased levels of C3 (n=54), and 6.0% of patients had decreased levels of C4 (n=29). Elevated IgA was a risk factor for the articular phenotype of BS (OR=2.652, P=0.011). Decreased complement C4 was a risk factor for the neurological phenotype of BS (OR=3.594, P=0.039). Positive ASO was a risk factor for the gastrointestinal phenotype of BS (OR=2.578, P=0.041). Elevated IL-6 was a risk factor for the ocular phenotype of BS (OR=7.560, P=0.016). Conclusion: HLA-B51 and AECA are common biomarkers in BS. Elevated IgA, decreased complement C4, positive ASO, and elevated IL-6 are risk factors for different phenotypes of BS.

Mimickers of nervous system involvement among patients with Behçet's syndrome.

We aimed to identify conditions mimicking nervous system involvement among patients with Behçet's syndrome (BS) and to determine clinical, laboratory and imaging findings that may help in the differential diagnosis. We screened the charts of 500 consecutive BS patients to identify those who were referred to neurology at any time during their follow-up. The final diagnoses, presenting signs and symptoms, laboratory and imaging results were retrieved from patient charts. Patients who did not have a follow-up visit during the last 3months were invited to the clinic. Among the 500 BS patients, 116 (23%) had been referred to neurology. Among these, 29 (5.8%) were diagnosed with typical central nervous system involvement of BS (NeuroBS). The type of NeuroBS was parenchymal involvement in 21 patients, cerebral venous sinus thrombosis in 7 patients, and both in 1 patient. 30 patients (6%) had other conditions related to the nervous system, 46 (9.2%) did not have a nervous system disorder, and their symptoms recovered spontaneously, and 11 (2.2%) were lost to follow-up without a definite diagnosis. Of the 30 BS patients who were diagnosed with another nervous system condition, 14 (46%) had primary headache syndromes, 6 (20%) had psychiatric disorders, 2 had entrapment neuropathy, and 1 each had epilepsy, glial tumor, multiple sclerosis, Meniere's disease, optic neuritis, neuroretinitis, steroid myopathy and polyneuropathy. Nervous system conditions other than NeuroBS are frequent among BS patients referred to neurology. Caution is required to avoid misdiagnosis of these patients as NeuroBS.

Profile of immunological biomarkers in Behcet’s syndrome: a large-scale single-center real-world study

Behcet's syndrome (BS) is a vasculitis characterized by immune dysregulation. Biomarkers are valuable for assessing clinically atypical pathogenesis. We aimed to investigate the distribution of different biomarkers and their effects on the clinical features of patients with BS in a large-scale, real-world study. This is a retrospective, single-center study. In total, 502 patients diagnosed with BS were enrolled in this study. We analyzed the clinical features of this cohort and divided patients’ symptoms into six categories, including mucocutaneous, articular, neurological, gastrointestinal, vascular, and ocular involvements. HLA-B51 cells, autoantibodies, and subsets of immune cells from the patients were tested. Pearson’s correlation, Wilcoxon rank sum test and multivariate logistic regression were used for data analysis. Various autoantibodies were detected in the serum of 40.8% of patients with BS. The positivity rate of anti-endothelial cell antibodies (AECA) was the highest among autoantibodies and was found in 23.5% (118/502) of patients with BS. The positivity rate of HLA-B51 in patients with BS was 27.1%. Tumor necrosis factor (TNF)-α, IL-2, and IL-4 producing CD4+ T cells were positively correlated with the gastrointestinal BS. Increased IL-4+CD4+ T cell was a risk factor for gastrointestinal BS (P = 0.006, Overall rate [OR] = 2.491, 95% Confidence interval [CI]: [1.317, 5.100]). Various autoantibodies can be detected in patients with BS. HLA-B51 and AECA are the most common biomarkers. Increased IL-4+ CD4+ T cell was a risk factor for gastrointestinal involvement in BS.

Long-term outcomes of infliximab treatment in neuro-Behcet syndrome: A single-center retrospective study.

Behcet's syndrome is a rare inflammatory disorder characterized by oral and genital ulcers, skin lesions, and uveitis. It exhibits a higher prevalence along the historic Silk Road. Neuro-Behcet syndrome (NBS) affects the central nervous system and poses significant morbidity and mortality risks. Infliximab, a TNF-alpha antagonist, has shown potential in NBS management, although the current evidence is mainly derived from case series due to the lack of randomized controlled trials. This retrospective study aimed to evaluate the disease outcomes during the first and second years following infliximab treatment in NBS patients experiencing attacks despite prior conventional immunosuppressive therapy. The study also sought to investigate the safety profile and adverse effects associated with infliximab. Fifty-three NBS patients were examined, with 22 receiving infliximab as either monotherapy or in combination with other therapies. Retrospective analysis was conducted on demographic data, clinical characteristics, and treatment responses. Treatment efficacy was measured using the Expanded Disability Status Scale (EDSS) modified for NBS. The study adhered to the Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) checklist guidelines. Among the study cohort, 60.4% had parenchymal NBS, and 39.6% had nonparenchymal NBS. Treatment with infliximab resulted in remission or disease stabilization in 95% of patients after one year and 68.7% after 2years. Relapse rates were 4.5% at 1year and 18.7% at 2years, with disease progression observed in two cases. Adverse effects were primarily mild to moderate, with no reports of serious adverse events. Infliximab exhibited efficacy in achieving remission or stabilization in NBS patients, maintaining a favorable safety profile. The timing of infliximab treatment may prevent the accumulation of disability and hinder disease progression. Nonetheless, future prospective studies are necessary to confirm these findings and refine treatment strategies for this complex condition.

De novo manifestations during adalimumab treatment in Behçet's syndrome.

Treatment response may be variable across organ manifestations of Behçet syndrome (BS). We aimed to determine the frequency of de novo manifestations during adalimumab treatment. We conducted a chart review of all BS patients who received adalimumab in our center between 2008 and 2023. Demographic data, reasons for initiating adalimumab, concurrent medications, previous treatments, and outcomes were recorded. We defined de novo manifestations as new BS manifestations that occurred for the first time during treatment with adalimumab. For patients with vascular involvement, a new vascular event at another vessel was also considered as a de novo manifestation. Among the 335 patients, a de novo manifestation developed in 14 (4%) patients. De novo manifestations were vascular involvement in 5 patients, arthritis in 3, anterior uveitis in 2, nervous system involvement in 2, gastrointestinal involvement in 1, and epididymitis in 1 patient. The primary reasons for adalimumab treatment were vascular involvement in 5 patients, uveitis in 4, arthritis in 3, mucocutaneous involvement in 1, and epididymitis in 1 patient. Upon the development of de novo manifestation, adalimumab was switched to another biologic in 4 patients, dose was intensified in 3, colchicine, conventional immunosuppressives, and/or glucocorticoids were added in 5, and topical eye drops were added in 2 patients, leading to remission of de novo manifestations in all patients. De novo manifestations were infrequent (4%) among BS patients treated with adalimumab. Of these, 57% were major organ involvement, mainly vascular involvement. None of the patients developed posterior uveitis.

Brain white matter microstructural alterations in Behcet's syndrome correlate with cognitive impairment and disease severity: A diffusion tensor imaging study

ObjectivesTo evaluate the microstructural integrity of brain white matter tracts in patients with Neuro-Behcet's syndrome (NBS) and Behcet's syndrome (BS) without neurological manifestations using diffusion tensor imaging (DTI) and to investigate potential utility of DTI as a surrogate biomarker of neurocognitive functioning and disease severity. MethodsThis cross-sectional study comprised 34 NBS patients and 32 BS patients without neurological involvement, identified based on the International Study Group of the Behcet's disease (ISGBD) and the International Consensus Recommendation (ICR) criteria, as well as 33 healthy controls. Cognitive functions, including attention, memory, language, abstraction, executive control, visuospatial skills, and sensorimotor performance were assessed using standardized questionnaires. DTI data were analyzed using tract-based spatial statistics (TBSS) and automated probabilistic tractography to investigate inter-group differences. Subsequently, correlations between tensor-derived parameters of white matter tracts, neurocognitive test scores, and disease severity measures were examined. ResultsDTI revealed decreased fractional anisotropy and increased radial diffusivity, mean diffusivity, and axial diffusivity in both supratentorial and infratentorial white matter in NBS patients, indicating widespread loss of microstructural integrity. Moreover, this loss of integrity was also observed in BS patients without neurological manifestations, albeit to a lesser extent. In NBS patients, certain white matter tracts, including cingulum bundle, were associated with poor cognitive performance across multiple domains and disease severity. DiscussionDTI findings might potentially serve as a neuroimaging marker to predict the extent of neurocognitive impairment and disease severity associated with central nervous system involvement in BS.

Impact of celiac disease in Behçet's syndrome patients: a study based on the database of Türkiye.

Our primary aim was to investigate the effects of concomitant celiac disease (CD) on the clinical characteristics of Behçet's syndrome (BS) patients. The study was a retrospective, nationwide, multicenter study. Turkish Ministry of Health National Electronic Database (e-Nabız) is used under Health Ministry's supervision to extract the subject's data. Statistical analyses were made by the Statistical Package for Social Sciences (SPSS) software version 20 (IBM Corp., Armonk, New York). Continuous variables were presented by mean ± standard derivation (SD) or median (min-max) according to normality and compared by student-t test. A binary logistic regression analysis was performed to further investigating the relation between having a concomitant CD with each BD manifestation and comorbidity, frequencies of which were detected to be significantly different in the student-test. A total of 84,241 patients diagnosed with BS were analyzed, and CD was identified in 175 (0.21 %) patients. The group with CD had a mean age of 41.30 ± 13.69 which was significantly younger. the prevalence of females was significantly higher (71.4%). The mean age of first admission for BS was also significantly younger in the group with CD (36.64 ± 13.28). BS patients with CD had a significantly higher prevalence of inflammatory bowel disease (27.2% vs. 7.3%, p < 0.001). When comorbid conditions were investigated depression (35.4% vs. 23.3%, p < 0.001), migraine (7.4 % vs. 2.6%, p < 0.001), fibromyalgia (10.9% vs. 4.5%, p < 0.001) and osteoporosis (12.6% vs. 6.6%, p = 0.001) were significantly more frequent in BS patients with CD. Our results suggest coexistence of CD in BS patients is related to female dominance and probably to an earlier disease onset. Several CD-related comorbidities as well as inflammatory bowel disease were more frequent in the CD group which implied an increased overall disease burden.

Gender disparities in Behçet's syndrome: identifying distinct phenotypes through cluster analysis.

Behçet's syndrome (BS) is a complex, multi-systemic disorder with a global occurrence, notably concentrated along the Silk Road. This study aimed to investigate gender-specific expressions and clinical phenotypes in BS patients within the Eastern Black Sea Region of Turkey. A total of 290 BS patients were retrospectively analyzed between January 2013 and December 2023. Demographic characteristics, clinical manifestations, medical treatment, and pathergy test results were obtained from a review of medical records. The mean age was 45.79 ± 13.05, with a male-to-female ratio of 48.6:51.4. Male patients had higher papulopustular lesions (p < 0.001) and ocular involvement (p = 0.036), while females showed more frequent genital ulcers (p = 0.032). Medication usage showed gender-based variations, notably higher corticosteroid, azathioprine, and tumor necrosis factor-alpha inhibitor (TNFi) use in males (p < 0.001). Cluster analysis revealed five distinct clusters, each with unique features and gender predominance. Cardiovascular type, ocular type, and skin type predominantly featured male patients, while joint involvement type and neurologic and mucosal involvement type were more prevalent among female patients with BS. This research contributes valuable insights into the gender-related clinical variations of BS within a specific geographic region, fostering a more comprehensive understanding of this challenging syndrome. The identification of distinct clinical phenotypes facilitates the development of tailored treatment strategies, potentially leading to improved outcomes for patients with BS.