UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.

UNASSIGNED: There is no specific marker that can be applied to determine the severity of Behçet\'s disease. The aim of this study is to investigate the potential of C-reactive protein (CRP)/albumin (CAR) ratio as a tool for assessing the severity of Behçet\'s disease.
UNASSIGNED: A retrospective crosssectional study was conducted by examining hospital archives. The CRP and albumin levels of Behçet\'s disease patients who presented to our dermatology clinic over a three-year period from February 2020 to February 2022 were included, along with the identical laboratory parameters in the control group. The CAR ratio was calculated and statistically compared across different clinical features of the disease and with the control group.
UNASSIGNED: Of the 97 patients with Behçet\'s disease, 70.1% (n = 68) were female and the median age was 36.0 years (IQR = 20.5), whereas of the 53 control subjects, 77.4% (n = 41) were female and the median age was 35.0 years (IQR = 19.0). There was no statistically significant difference in sex or age between the groups (p > 0.05). The levels of CRP and CAR were found to be significantly elevated in patients with Behçet\'s disease compared to the control group (both p < 0.001). According to the ROC analysis, the area under the curve (AUC) of CRP level and CAR were found to be 0.784 (95%CI: 0.710-0.859), and 0.786 (95%CI: 0.712-0.861), respectively. The cut-off value for CRP was determined as 1.485, whereas for CAR it was 0.324. As the severity of Behçet\'s disease increased, there was a statistically significant increase in the CAR level (p < 0.001). The severity of Behçet\'s disease was statistically significantly associated with a high CAR level.
UNASSIGNED: CAR can be used as a quick and easily calculated parameter to assess the severity of Behçet\'s disease.

UNASSIGNED: Syphilis exhibits a wide range of clinical presentations, mimicking various systemic and ocular diseases. Ocular syphilis, in particular, manifests with multiple presentations, ranging from anterior uveitis to panuveitis, making it a potential differential diagnosis for Behçet\'s uveitis. Here, we present a unique case of Behçet\'s panuveitis that was undergoing immunomodulatory therapy and was complicated by ocular syphilis. Notably, this case also featured rare cutaneous manifestations associated with secondary syphilis, commonly known as malignant syphilis.
UNASSIGNED: A 38-year-old Thai man with refractory end-stage Behçet\'s panuveitis reported a maculopapular rash accompanied by increased intraocular inflammation. The escalation of immunomodulatory therapy, intended to manage the provisional diagnosis of active ocular and cutaneous Behçet\'s disease, resulted in clinical deterioration, with the rash transforming into multiple noduloulcerative lesions. Despite negative serologic tests for syphilis at baseline before initiating immunomodulatory therapy, syphilis infection was eventually diagnosed following reevaluation and the observation of spirochetes in a skin biopsy specimen. The patient was treated with intravenous penicillin G, resulting in an improvement in intraocular inflammation and resolution of noduloulcerative rashes.
UNASSIGNED: Intraocular inflammation and mucocutaneous lesions in syphilis can mimic the presentation of Behçet\'s disease. The introduction of immunomodulatory therapy may alter the clinical picture, leading to a severe and atypical presentation. A high index of suspicion for reevaluating serologic tests or performing tissue biopsies is warranted for an accurate diagnosis.

Behçet\'s Disease (BD), also recognized as Behçet Syndrome, manifests uniquely in pediatric populations as Pediatric Behçet\'s Disease (PBD), characterized by multisystemic inflammatory symptoms including recurrent oral and genital aphthae, and diverse ocular, vascular, and neurological involvements. This review elucidates the prevalence, burden, and management strategies of headaches in children with PBD, focusing on both primary headaches, such as migraine and tension-type headaches, and secondary headaches linked to systemic disease manifestations. It explores the pathophysiological underpinnings specific to PBD-related headaches and discusses the intricate relationship between systemic inflammatory processes and neurological symptoms. By examining the literature from 2004 to 2024, this study highlights the high frequency of headache in PBD patients, underscoring its diagnostic and clinical significance. We aim to provide a detailed understanding of headache management in PBD, emphasizing tailored therapeutic strategies that address the unique challenges faced by this patient population. This review also underscores the importance of comprehensive clinical evaluations to optimize outcomes and mitigate long-term sequelae, proposing that awareness and understanding of headache in PBD can significantly enhance both diagnosis and management.

Behçet\'s disease (BD) is a multifaceted autoimmune disorder affecting multiple organ systems. Vascular complications, such as venous thromboembolism (VTE), are highly prevalent, affecting around 50% of individuals diagnosed with BD. This study aimed to identify potential biomarkers for VTE in BD patients. Three microarray datasets (GSE209567, GSE48000, GSE19151) were retrieved for analysis. Differentially expressed genes (DEGs) associated with VTE in BD were identified using the Limma package and weighted gene co-expression network analysis (WGCNA). Subsequently, potential diagnostic genes were explored through protein-protein interaction (PPI) network analysis and machine learning algorithms. A receiver operating characteristic (ROC) curve and a nomogram were constructed to evaluate the diagnostic performance for VTE in BD patients. Furthermore, immune cell infiltration analyses and single-sample gene set enrichment analysis (ssGSEA) were performed to investigate potential underlying mechanisms. Finally, the efficacy of listed drugs was assessed based on the identified signature genes. The limma package and WGCNA identified 117 DEGs related to VTE in BD. A PPI network analysis then selected 23 candidate hub genes. Four DEGs (E2F1, GATA3, HDAC5, and MSH2) were identified by intersecting gene sets from three machine learning algorithms. ROC analysis and nomogram construction demonstrated high diagnostic accuracy for these four genes (AUC: 0.816, 95% CI: 0.723-0.909). Immune cell infiltration analysis revealed a positive correlation between dysregulated immune cells and the four hub genes. ssGSEA provided insights into potential mechanisms underlying VTE development and progression in BD patients. Additionally, therapeutic agent screening identified potential drugs targeting the four hub genes. This study employed a systematic approach to identify four potential hub genes (E2F1, GATA3, HDAC5, and MSH2) and construct a nomogram for VTE diagnosis in BD. Immune cell infiltration analysis revealed dysregulation, suggesting potential macrophage involvement in VTE development. ssGSEA provided insights into potential mechanisms underlying BD-induced VTE, and potential therapeutic agents were identified.

UNASSIGNED: While observational epidemiological studies have suggested an association between gut microbiota and Behçet\'s disease (BD), the causal relationship between the two remains uncertain.
UNASSIGNED: Statistical data were obtained from gut microbiome Genome-Wide Association Studies (GWAS) published by the MiBioGen consortium, and genetic variation points were screened as instrumental variables (IV). Mendelian randomization (MR) study was performed using inverse variance weighted (IVW), weighted median, MR-Egger regression, simple mode, and weighted mode methods to evaluate the causal relationship between gut microbiota (18,340 individuals) and BD (317,252 individuals). IVW was the main method of analysis. The stability and reliability of the results were verified using the leave-one-out method, heterogeneity test, and horizontal genetic pleiotropy test. Finally, a reverse MR analysis was performed to explore reverse causality.
UNASSIGNED: Inverse variance weighted (IVW) results showed that the genus Parasutterella (OR = 0.203, 95%CI 0.055-0.747, p = 0.016), Lachnospiraceae NC2004 group (OR = 0.101, 95%CI 0.015-0.666, p = 0.017), Turicibacter (OR = 0.043, 95%CI 0.007-0.273, p = 0.001), and Erysipelatoclostridium (OR = 0.194, 95%CI 0.040-0.926, p = 0.040) were protective factors against BD, while Intestinibacter (OR = 7.589, 95%CI 1.340-42.978, p = 0.022) might be a risk factor for BD.
UNASSIGNED: Our study revealed the causal relationship between gut microbiota and BD. The microbiota that related to BD may become new biomarkers; provide new potential indicators and targets for the prevention and treatment of BD.

A 53-year-old woman with recurrent stomatitis, genital ulcers, and folliculitis was admitted to our hospital after experiencing visual disturbances for the past two weeks, and a non-throbbing headache for the past three days. She had also developed numbness in her left extremities. An ophthalmological examination revealed inflammatory changes in the eye. Cerebrospinal fluid analysis showed increased cell counts, protein, and interleukin-6 levels. Brain magnetic resonance imaging revealed multiple high signal intensities on T2-weighted (T2W)/fluid-attenuated inversion recovery (FLAIR) images of the pons and occipital and parietal lobes. The T2W/FLAIR high-signal-intensity lesion in the pons was hyperintense on diffusion-weighted imaging (DWI) and hypointense on apparent diffusion coefficient mapping (ADC), suggesting cytotoxic edema. Another high-signal-intensity lesion on T2W/FLAIR was isointense to hyperintense on DWI and hyperintense on ADC, indicating vasogenic edema. The vasogenic edema in the left occipital lobe contained a small core that was hyperintense on DWI and hypointense on ADC, suggesting cytotoxic edema. The patient was diagnosed with acute neuro-Behçet\'s disease (neuro-BD) and responded well to high-dose glucocorticoid and colchicine treatment. The present report emphasizes that patients with acute neuro-BD may present with cytotoxic edema in the pons and cerebral spheres. Further reports of similar cases would contribute to a better understanding of the role of cytotoxic edema in the pathophysiology of neuro-BD and help elucidate the mechanisms underlying a unique presentation characterized by a central cytotoxic edema core within vasogenic edema. (233 words).

OBJECTIVE: Oral and genital ulcers are the hallmark manifestation of Behçet\'s disease (BD), significantly impacting patients\' quality of life. Our study focuses on comparing the effectiveness and safety of TNF inhibitors (TNFis) and apremilast in controlling oral ulcers of BD, aiming to provide evidence-based guidance for physicians in selecting appropriate treatment modalities.
METHODS: A retrospective analysis was performed on BD patients treated between December 2016 and December 2021 with TNFis or apremilast for refractory oral ulcers. The study assessed treatment response by the absence of oral ulcers at 3 and 6 months, with additional evaluations for genital ulcers and articular involvement.
RESULTS: The study included 78 patients, equally allocated between TNFis and apremilast treatments. Both groups showed significant oral ulcer reduction at 3 (p< 0.001) and 6 months (p= 0.01) with no significant difference between the treatments. Apremilast had a notable corticosteroid-sparing effect by the 3-month follow-up, persisting through 6 months. Both treatments were equally effective in reducing genital ulcers, with TNFis showing greater effectiveness in addressing articular involvement. Apremilast had a higher discontinuation rate due to gastrointestinal side effects.
CONCLUSIONS: TNFis and apremilast are both effective for treating BD refractory oral ulcers. While TNFis may offer broader benefits for other disease manifestations, apremilast is distinguished by its corticosteroid-sparing effect, especially for patients with a milder disease phenotype. Treatment selection should consider individual disease severity and clinical features to ensure a personalized and effective management strategy.

Behçet\'s disease is a chronic systemic inflammatory vasculitis of unknown etiology. It is characterized by recurrent episodes of oral aphthous ulcers, genital ulcers, skin lesions, ocular lesions, and other manifestations. This disease affects many organs and systems, showing a wide range of clinical features. Although pulmonary artery involvement is not common in Behçet\'s disease, its presence carries a substantial risk of mortality. This report provides a detailed history of a 25-year-old male who was admitted with productive cough, hemoptysis, dyspnea on minimal exertion, fever, and chest pain. He had recurrent orchitis and epididymitis for 7 years, as well as oral and genital ulcers and severe headache. Clinical examination revealed decreased breath sounds at the right middle lung. Thoracic computed tomography angiography confirmed multiple pulmonary artery aneurysms bilaterally. The patient was diagnosed with Behçet\'s disease, and immunosuppression therapy was initiated. During follow-up, the patient did not report any complications. This case report underscores the significance for clinicians to consider Behçet\'s disease as a differential diagnosis in patients presenting with hemoptysis and a history of orchitis and epididymitis, given that Behçet\'s disease rarely causes pulmonary artery aneurysms.

UNASSIGNED: The treatment of Behçet\'s disease has improved significantly with the introduction of biologic therapies. However, there is still a need for more information about their use. This study aimed to evaluate the indications, response, and side effects of biologic agents in patients with refractory or severe Behçet\'s disease in the south of Iran, their follow-up and reasons for changing the biologics.
UNASSIGNED: A retrospective analysis was conducted on 44 patients aged 16-65 years who were prescribed biologic agents for at least 6 months. The clinical history, partial and complete remission at 6 and 12 months, occurrence of side effects, and need for switching to a second or third biologic agent were recorded.
UNASSIGNED: The most common indications for starting biologic agents were ophthalmic (68.2%), parenchymal brain involvement (15.9%), and arthritis (11.4%). Improvement was observed in various manifestations of Behçet\'s disease, with complete remission in 86, 51.6, 92.8, 66.7, 42.9, 33.3, and 80.0% of oral aphthous lesions, ophthalmic activity, genital aphthous lesions, skin activity, arthritis, brain parenchymal lesions, and vascular activity, respectively, 6 months after starting biologic agents. These rates were unchanged or increased at the 12-month follow-up. In 25.0% of patients, a switch to a second biologic agent was necessary due to severe disease, side effects, or refractory disease. Side effects occurred in 16.3% and 33.3% of patients on the first and second biologic agents, respectively. The majority of side effects were not serious.
UNASSIGNED: We found a promising improvement at 6-month and 12-month follow-ups with various biologic agents in treating Behçet\'s disease with an acceptable safety profile.