Search

The current study was deployed to evaluate the role of metastasis-associated lung adenocarcinoma transcript 1 (MALAT1) and miR-155, along with the inflammatory markers, TNFα and IL-6, and the adhesion molecule, cluster of differentiation 106 (CD106), in Behçet's disease (BD) pathogenesis. The study also assessed MALAT1/miR-155 as promising diagnostic and prognostic biomarkers for BD. The current retrospective case–control study included 74 Egyptian BD patients and 50 age and sex-matched controls. Blood samples were collected, and then, serum samples were separated for further biochemical and molecular investigations. The gene expression of MALAT1 and miR-155 was measured using qRT-PCR, whereas the levels of TNFα, IL-6, and CD106 were estimated using ELISA technique. MALAT1 was significantly downregulated, whereas miR-155 was upregulated among BD patients, compared with control subjects. Levels of TNFα, IL-6, and CD106 were elevated in BD patients. Further downregulation in MALAT1 together with upregulation of miR-155 was observed in active BD patients, relative to the inactive group. Receiver-operating-characteristic analysis revealed that MALAT1 and miR-155 could discriminate BD patients from controls, on the one hand, and active from inactive BD patients, on the other hand. MALAT1 was negatively correlated with TNFα, IL-6, and CD106, while miR-155 was positively correlated with them. Logistic regression analyses demonstrated miR-155 as a significant independent predictor of BD susceptibility, and MALAT1 as an independent negative predictor of BD activity. For the first time, the current research enlightens the role of MALAT1 and miR-155 in BD pathogenesis via impacting IL-6/TNF-α/CD-106 signaling. As well, MALAT1 and miR-155 could be regarded as novel non-invasive biomarkers that may improve BD diagnosis and prognosis. Key Points •MALAT1/miR-155 exerts potential role in Behçet's disease. •MALAT1/miR-155 are promising biomarkers for Behçet's disease. •MALAT1/miR-155 targets IL-6/TNF-α/CD-106 signaling. [ABSTRACT FROM AUTHOR]

Purpose: The role of fibroblast activation protein (FAP)-targeted imaging in systemic vasculitis is currently unclear. We aimed to evaluate the clinical value of fluorine-18-labeled FAP inhibitor 42 ([18F]FAPI-42) in patients with systemic vasculitis and to compare with [18F]fluorodeoxyglucose (FDG) imaging. Methods: Patients with systemic vasculitis who underwent dual-tracer PET/CT([18F]FDG and [18F]FAPI) imaging from September 2020 to March 2022 were retrospectively analyzed. Positive lesions are defined as vascular/extravascular lesions with increased tracer uptake above surrounding background, which cannot be attributed to the physiologic biodistribution of the radiotracer. The vascular/extravascular lesion detection rate and semiquantitative values (SUVmax, TBRblood and TBRliver) of [18F]FAPI and [18F]FDG were compared, and the correlation between the extent and range of tracer uptake and levels of inflammatory markers was investigated. Results: Thirty patients (13 males and 17 females; mean age, 52.5 ± 17.2 years) with systemic vasculitis were included (17 large vessel vasculitis, 10 anti-neutrophil cytoplasmic antibody-associated vasculitis, 2 Behcet's disease and 1 polyarteritis nodosa). [18F]FDG PET/CT had positive findings in 93.3% (28/30) of patients, while [18F]FAPI PET/CT had positive findings in all patients (100%, P = 0.500). Compared with [18F]FDG PET/CT, [18F]FAPI PET/CT detected more lesions (161/168 vs. 145/168, P = 0.005), and more extensive vascular involvement in 60% (18/30) of patients. Although SUVmax did not differ significantly between [18F]FAPI and [18F]FDG (median, 5.94 vs. 5.46, P = 0.517), [18F]FAPI had higher TBRliver (median, 9.59 vs. 3.15, P < 0.001) and TBRblood (median, 5.45 vs. 4.20, P = 0.006). The total number of positive lesions in FAPI PET/CT show a moderate correlation with erythrocyte sedimentation rate (rs =0.478, P = 0.008) and C-reactive protein (rs =0.486, P = 0.006). After treatment, follow-up FAPI PET/CT of 6 patients showed decreased SUVmax, TBR and number of detected lesions, paralleling the clinical remission. Conclusion: [18F]FAPI PET/CT imaging is a promising imaging modality for the diagnosis and therapeutic monitoring of systemic vasculitis. [ABSTRACT FROM AUTHOR]

Background: Behçet's disease (BD) is a common disease with high morbidity and mortality. Ocular, neurological, and vascular affection in BD are a major cause of such morbidity and mortality. Evoked potential (EP) studies including visual evoked potentials (VEP) and somatosensory evoked potentials (SSEP) in addition to antiphospholipid antibodies (aPL) are valuable tools for detection of ocular, neurological and vascular affection and may be used for assessment of activity and severity in BD. We aimed to use EP studies and aPL as objective tools in BD assessment. Results: Abnormal VEP was found in 76% of BD patients and has a significant association with ocular involvement (P = 0.003), Moreover, Abnormal VEP was significantly associated with ocular activity (P = 0.002). Abnormal SSEP in both median and tibial nerves was found in 62% of patients, prolonged central conduction time of median and tibial nerves was found in 46% and 22% of patients respectively and was significantly associated with neurological involvement in BD (P < 0.05). Abnormal VEP and SSEP studies were significantly associated with BD disease severity (P < 0.05) but not activity. Vascular involvement was significantly associated with elevated lupus anticoagulant and anti-cardiolipin IgM antibodies (P < 0.05), elevated lupus anticoagulant levels were associated with optic nerve involvement and abnormal VEP. Conclusion: Abnormal VEP and SSEP studies can be considered as indicators for BD severity and hence poorer prognosis. VEP study is a valuable tool in BD and may indicate ocular involvement or ocular activity. Prolonged Central conduction time of upper limb and lower limb evaluated by N13- N20 IPL and N22-P39 IPL respectively may be used to detect nervous system involvement in BD patients. Optic nerve involvement may be suspected in BD patients with elevated lupus anticoagulant, elevated LA and aCL IgM can be used as indicators for vascular involvement in BD. [ABSTRACT FROM AUTHOR]

Background: Behçet's disease (BD) during pregnancy is a relatively rare condition, and there are currently no established guidelines for its management. The effects of BD on both mothers and children remain unclear. In this paper, we present the diagnostic and treatment processes for a patient with BD during pregnancy. Case presentation: A 20-year-old woman, gravida 2, para 1, presented to our inpatient department with recurrent oral and genital ulcers at 28 weeks and 3 days of gestation. Given the complexity of the patient's condition, we initiated the first multidisciplinary consultation to establish appropriate treatment regimens. However, the initial treatment was ineffective, and the patient experienced a fever for four days. Consequently, we conducted a second multidisciplinary consultation. The final treatment regimen included azithromycin, hydroxychloroquine, prednisone, and low-molecular-weight heparin calcium. The clinical symptoms resolved, and the female neonate was delivered successfully. No relapse was observed during the four-month follow-up. Conclusions: The critical issues in treatment involve ensuring medication safety for pregnant women and preventing adverse pregnancy outcomes. Notably, one potential cause of adverse pregnancy outcomes in individuals with BD is vasculopathy, which necessitates prompt treatment with anticoagulant therapy. Obstetricians should closely monitor disease progression and remain vigilant for complications in order to minimize adverse outcomes during pregnancy. [ABSTRACT FROM AUTHOR]

Numerous studies have investigated the alterations of genes, proteins, and metabolites in Behcet's disease (BD). By far, little is known about the depiction of panoramic changes underlying this disease. This study purposed to assess the consistently dysregulated genes, proteins, and metabolites in BD across publications using the vote-counting approach. This study was based on 745 studies that identified a collection of 2354 differential molecules with 3574 molecule entries in blood, peripheral blood mononuclear cells, CD4+T cells, and aqueous humor samples from patients with BD. In this study, the results of binomial analysis showed that the circulating levels of 38 molecules, including interferon-γ and interleukin 17, were consistently upregulated, whereas high density lipoprotein cholesterol, apolipoprotein A, hemoglobin, and glutathione were consistently downregulated. The levels of interferon-γ, tumor necrosis factor-α and toll like receptor 4 in peripheral blood mononuclear cells, and interferon-γ and interleukin-17 in CD4+T cells were consistently upregulated. Additionally, the levels of interferon-γ, tumor necrosis factor-α and C-X-C motif chemokine ligand 8 in aqueous humor were consistently upregulated. Collectively, this study showed that the hyperactivity of Th1 and Th17 responses played an essential role in the pathogenesis of BD, and the progression of this disease was associated with enhanced neutrophil chemotaxis, vascular endothelial injury, activation of haemostatic system, and oxidative stress. [ABSTRACT FROM AUTHOR]

Background: Intestinal Behçet's syndrome (IBS) has high morbidity and mortality rates with serious complications. However, there are few specific biomarkers for IBS. The purposes of this study were to investigate the distinctive metabolic changes in plasma samples between IBS patients and healthy people, active IBS and inactive IBS patients, and to identify candidate metabolic biomarkers which would be useful for diagnosing and predicting IBS. Methods: In this study, we performed a global untargeted metabolomics approach in plasma samples from 30 IBS patients and 20 healthy subjects. P value < 0.05 and variable importance projection (VIP) values > 1 were considered to be statistically significant metabolites. Univariate receiver operating characteristic (ROC) curve analysis was plotted as a measure for assessing the clinical performance of metabolites, and area under curve (AUC) were assessed. Results: A total of 147 differentially abundant metabolites (DAMs) were identified between IBS patients and normal control (NC) group. The potential pathways involved in the pathogenesis of IBS include linoleic acid metabolism; GABAergic synapse; biosynthesis of unsaturated fatty acids; valine, leucine and isoleucine biosynthesis; ovarian steroidogenesis; and others. In addition, a total of 103 significant metabolites were selected to distinguish active IBS from inactive IBS patients. Tyrosine metabolism, dopaminergic synapse and neuroactive ligand-receptor interaction were found to be closely related to the disease activity of IBS. Furthermore, three potential metabolites including quinate, stearidonic acid (SDA) and capric acid (CA) could significantly differ IBS patients from NC group. On the other hand, 1-methyladenosine (m1A), genipin, methylmalonic acid (MMA) and ascorbate could significantly differentiated active IBS from inactive IBS patients. Conclusion: In conclusion, this study demonstrated the characteristic plasma metabolic profiles between IBS group and NC group, as well as between active and inactive IBS patients by using an untargeted LC/MS metabolomics profiling approach. In this study, quinate, SDA and CA were identified as potential diagnostic biomarkers for IBS. Additionally, m1A, genipin, MMA and ascorbate could serve as potential biomarkers for evaluating IBS activity. These findings might provide potential valuable insights for developing therapeutic strategies to manage IBS in the future. [ABSTRACT FROM AUTHOR]

Background: Behçet's disease (BD) is a multisystem inflammatory disorder that can affect various organs, including the lungs. Pulmonary manifestations are rare and typically present as pulmonary artery aneurysms. Case presentation: We report the case of a 56-year-old East Asian male with a 27-year history of BD, who had no respiratory symptoms, such as hemoptysis, cough, or fever. Chest imaging revealed lymph node enlargement and a lung mass, initially raising concerns of malignancy. Despite the suspicious radiological findings, three biopsies, including a surgical biopsy, were all negative for malignancy. Remarkably, over the course of 2 years, these pulmonary manifestations spontaneously regressed without any specific treatment for lung involvement, and the patient remained in a stable BD state. Conclusions: BD patients are at an increased risk for malignancy compared to healthy controls, making it crucial to differentiate between malignancy and BD-related pulmonary abnormalities. This case highlights that, in the absence of active BD symptoms, lymphadenopathy and lung masses can occur and may resolve spontaneously. Accurate diagnosis and vigilant monitoring are essential in BD patients but BD-related lung involvement should be considered in similar cases. [ABSTRACT FROM AUTHOR]

Background: Recent studies have highlighted the potential role of several long non-coding RNAs (lncRNAs) in the pathogenesis of Behçet's disease (BD). This study investigated the expression profiles of lncRNA NEAT1 and lncRNA HOTAIR, and their target cytokine genes, IL-6 and TNF-α, in active and inactive BD patients. Methods: This cross-sectional study was conducted on peripheral blood mononuclear cells (PBMCs) obtained from 25 BD patients and 25 age-sex-matched healthy controls (HCs). BD activity was evaluated using the BDCAF (Behcet's Disease Current Activity Form) score. Correlation analysis assessed the relationship between BD activity and the expression levels of lncRNAs and their target cytokine genes. The diagnostic potential of the genes was evaluated using Receiver Operating Characteristic (ROC) curve analysis. Results: The expression levels of NEAT1, HOTAIR, IL-6, and TNF-α were significantly higher in the BD group compared to the HCs. However, there was no significant correlation between the expression levels of these genes and BD activity or the involvement of various organs. A positive correlation was observed between HOTAIR gene expression and IL-6 (R = 0.594, P-value = 0.009) in the BD group. In contrast, no significant correlation was found between HOTAIR and TNF-α or between NEAT1 and TNF-α or IL-6. The ROC curve analysis indicated strong diagnostic potential for the lncRNAs, with area under the curve (AUC) values of 0.90 for NEAT1 and 0.86 for HOTAIR. Conclusion: The elevated expression levels of NEAT1 and HOTAIR in BD patients suggest their potential involvement in the disease's pathogenesis, indicating promising targets for future diagnostic and therapeutic strategies in BD. [ABSTRACT FROM AUTHOR]

Purpose: To explore if COVID-19 infection and its subsequent immunosuppressant adjustment as well as previous vaccination status are associated with higher risks of uveitis flare in patients with Behcet's disease. Methods: This retrospective multicenter cohort study was conducted in January 2023 among patients with Behcet's uveitis, during the second wave of the COVID-19 pandemic in China, with an anticipated sample size of 250. The primary objective was to examine the association between COVID-19 infection and the occurrence of uveitis flare. The potential impact of other exposures, including the patient's vaccination status and treatment adjustments to the risk of uveitis flare and the course of COVID-19 infection were also analyzed. Results: 207 patients with COVID-19 infection and 47 patients without COVID-19 infection were included. A total of 127 uveitis flares occurred in the observational period (14.29 events per 100 person-month). COVID-19 infection was found to be significantly associated with a higher rate of uveitis flare (adjusted rate ratio = 4.8, 95% CI 3.7 to 6.3, P < 0.001). However, neither systemic immunosuppressive adjustment nor COVID-19 vaccination status showed a significant association with uveitis flare or the course of COVID-19 infection. Conclusions: This study provides evidence of an association between COVID-19 infection and an increased risk of uveitis flare in patients with Behcet's disease. However, there was no significant evidence to support that baseline immunosuppressive therapy regimens, treatment adjustment after COVID-19 infection, or vaccination status were associated with higher risks of uveitis flare or prolonged COVID-19 course. Key messages: What is known: • Case reports have suggested that COVID-19 might trigger uveitis flares in patients with Behcet's disease, but no clinical studies have confirmed this the potential association. What is new: • Our cohort study demonstrate that COVID-19 infection was significantly associated with higher uveitis flare rate in patients with Behcet's uveitis. • Uveitis flare was not associated with systemic immunosuppressive adjustment and patients' COVID-19 vaccination status. [ABSTRACT FROM AUTHOR]

Apremilast (Otezla®) is an inhibitor of phosphodiesterase-4 (PDE-4) being developed by Amgen. It is approved in multiple countries worldwide, including the USA and those of the EU, for the treatment of adults with psoriatic arthritis, plaque psoriasis, or oral ulcers associated with Behçet's disease. In April 2024, based on clinical data in patients aged 6–17 years, apremilast received its first pediatric approval in the USA for the treatment of pediatric patients aged ≥ 6 years and weighing ≥ 20 kg with moderate to severe plaque psoriasis who are candidates for phototherapy or systemic therapy. Apremilast was also approved in the EU in October 2024 for the treatment of moderate to severe plaque psoriasis in children and adolescents from the age of 6 years and weighing ≥ 20 kg who are candidates for systemic therapy. This article summarizes the milestones in the development of apremilast leading to the first pediatric approval for plaque psoriasis. [ABSTRACT FROM AUTHOR]

Background: Behcet's disease (BD) is a rare and recurrent autoinflammatory disorder characterized by systemic vasculitis, frequently manifested as recurrent aphthous stomatitis (RAS). We aim to identify specific serum proteins to discriminate between BD and idiopathicRAS. Method: Peripheral blood was collected from 12 BD patients, 12 idiopathic RAS patients, and 21 healthy volunteers. The serum samples underwent Tandem Mass Tag-based mass spectrometry analysis. Differentially expressed proteins (DEPs) were identified for KEGG pathway enrichment, Gene Ontology (GO), and protein-protein interaction (PPI) analyses. ELISA was utilized to verify two BD-specific DEPs in another cohort consisting of 18 BD patients, 18 idiopathic RAS patients, and 18 controls. Results: Compared with RAS serum, BD serum showed 242 DEPs. 49 proteins were differentially expressed in BD but not RAS serum compared to healthy controls. KEGG pathway and GO analyses revealed that DEPs in BD and RAS have similar biological functions and cellular distributions, featuring a significant association with pathways regulating blood coagulation and immune response. When comparing DEPs between BD and RAS, several keratins emerged as markers that distinguish RAS from BD. We also identified multiple DEPs in BD but not RAS patients. PPI analysis uncovered that lipoprotein metabolism regulators serve as hub proteins, indicating their potentially essential roles in BD pathology. In addition, ELISA results confirmed the elevated LRG1 and SOD3 levels in BD, but not RAS patients, compared to healthy donors. Conclusion: Our data uncovered novel serum proteins that distinguish BD from RAS, which may potentially be useful in BD diagnosis and treatment. [ABSTRACT FROM AUTHOR]

Background/Objectives: Recurrent aphthous stomatitis is a common oral mucosal disorder characterized by painful ulcerations and frequent recurrences, which can significantly impair quality of life. This study explores the efficacy of zinc-enriched multivitamin supplementation (ZnVita, containing 22.5 mg of elemental zinc) for the treatment of recurrent aphthous stomatitis in treatment-naïve patients, aiming to diminish the reliance on immunomodulatory drugs. Methods: Conducted as a retrospective observational study at a tertiary referral hospital from 2013 to 2023, we analyzed 201 patients who received ZnVita daily for a minimum duration of one month as their initial management. Patients who were using systemic immunomodulating agents or met the diagnostic criteria for Behçet's disease were excluded. Results: Of the 201 patients, 95 presented with an oral ulcer alone and 106 exhibited additional symptoms associated with Behçet's disease. Efficacy analysis was conducted on 155 patients due to follow-up loss or incomplete data. Among them, 58.7% (91/155) showed partial or significant responses. Patients with BD-related symptoms were significantly more prevalent among non-responders (64.1%, 41/64) compared to responders (42.9%, 39/91), with a statistically significant difference (p = 0.009). Treatment was well-tolerated, with mild gastrointestinal adverse events reported in only 2.5% of cases. Conclusions: These results suggest that zinc-enriched multivitamin supplementation offers a beneficial and safe initial treatment alternative for a considerable proportion of treatment-naïve recurrent aphthous stomatitis patients, especially those without concurrent symptoms of Behçet's disease, showcasing its potential in reducing the future need for immunomodulatory treatments. [ABSTRACT FROM AUTHOR]

Purpose: The clinical relevance of human leukocyte antigen (HLA) subtypes such as HLA-B51 on Behçet's disease (BD)-related uveitis and non-infectious uveitis (NIU) unrelated to BD remains largely unknown. Methods: Data were prospectively collected from the International AIDA Network Registry for BD and for NIU. We assessed differences between groups (NIU unrelated to BD and positive for HLA-B51, BD-related uveitis positive for HLA-B51 and BD-related uveitis negative for HLA-B51) in terms of long-term ocular complications, visual acuity (VA) measured by best corrected visual acuity (BCVA), anatomical pattern, occurrence of retinal vasculitis (RV) and macular edema over time. Results: Records of 213 patients (341 eyes) were analyzed. No differences in complications were observed (p = 0.465). With regard to VA, a significant difference was detected in median BCVA (p = 0.046), which was not maintained after Bonferroni correction (p = 0.060). RV was significantly more prevalent in NIU-affected patients who tested positive for HLA-B51, irrespective of the systemic diagnosis of BD (p = 0.025). No differences emerged in the occurrence of macular edema (p = 0.99). Conclusions: Patients with NIU testing positive for HLA-B51 exhibit an increased likelihood of RV throughout disease course, irrespective of a systemic diagnosis of BD. The rate of complications as well as VA are comparable between NIU cases unrelated to BD testing positive for HLA-B51 and uveitis associated with BD. Therefore, it is advisable to perform the HLA-B typing in patients with NIU or retinal vasculitis, even in the absence of typical BD features. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the serum asymmetric dimethylarginine (ADMA) level as a biomarker for uveitis in Behçet's Disease (BD). Methods: In this cross-sectional study, two groups of BD patients were examined: 33 with uveitis and 27 without uveitis. All patients were clinically evaluated, with disease activity measured by Behçet's Disease Current Activity Form (BDCAF) score. They also underwent thorough ophthalmic evaluation, and routine laboratory investigations, including serum ADMA. Results: Patients with BD who experienced active or inactive uveitis had higher levels of serum ADMA compared to those without uveitis. Anterior (ρ = 0.34, p < 0.01), posterior (ρ = 0.3, p < 0.05), and pan uveitis (ρ = 0.35, p < 0.01) were significantly correlated with serum ADMA levels. However, there was no significant correlation between ADMA and other BD manifestations. ROC curve analysis showed that increased serum ADMA levels in BD patients predicted uveitis with a sensitivity of 61.8%, specificity of 96.2%, and AUC of 0.78(95% CI: 0.66–0.9, p < 0.001). Conclusion: Serum ADMA level can serve as a novel biomarker of uveitis in BD and its severity with good diagnostic accuracy, regardless of its site or activity. [ABSTRACT FROM AUTHOR]

Purpose: Behcet's Disease is a chronic multisystem vasculitis associated with a blinding uveitis. Few comparative studies exist between conventional disease-modifying antirheumatic drugs (DMARDs) and biologic DMARDs in Behcet's uveitis (BU). We therefore used drug retention time (DRT), an accepted surrogate measure of pharmacological efficacy and tolerability, to compare these treatments in patients with BU. Methods: Retrospective chart review of patients who met the revised International Criteria for Behcet's Disease (ICBD) treated at the Royal Victorian Eye and Ear Hospital, Australia, between 1985–2021. DRT was analysed with Kaplan-Meier plots and defined as total time on drug in the first medication-period for each DMARD in each patient. Results: Forty-eight patients (37 males) with median age of 28.6 years were followed-up for a median of 8.0 years. At initial presentation, half had bilateral disease and median logMAR visual acuity was 0.176 (Snellen 6/9) in 62 uveitic eyes (16 anterior uveitis, 11 intermediate, 2 posterior, and 33 panuveitis). Thirty-three patients met ICBD initially. Prescribed corticosteroid-sparing agents were Cyclosporin (N = 24), Mycophenolate (N = 22), Azathioprine (N = 22), Methotrexate (N = 16), and Adalimumab (N = 15). Median DRT was 14.0, 27.4, 8.3, 24.0, and 52.0 months, respectively. DMARDs were discontinued 116 times and adverse effects (N = 37) were the main reason for cessation. Over time, patients were switched from Cyclosporin to Adalimumab earlier in the disease course due to poorer tolerance of adverse events. Conclusion: Adalimumab's drug retention time was found to be similar to and possibly better than cDMARDs in patients with BU, who often suffer from vision-threatening disease at first presentation. [ABSTRACT FROM AUTHOR]

Phosphodiesterase-4 (PDE4) is involved in the synthesis of inflammatory cytokines that mediate several chronic inflammatory disorders, including psoriasis and atopic dermatitis. In recent years, the therapeutic armamentarium in dermatology has expanded with the introduction of PDE4 inhibitors, both in oral and topical formulations. PDE4 inhibitors have gained increasing interest due to their remarkable safety record and ease of prescription, as evidenced by the recent influx of literature detailing its off-label uses. Apremilast was the first PDE4 inhibitor approved by the US Food and Drug Administration (FDA) and the European Medicines Agency (EMA) for psoriasis, psoriatic arthritis, and oral ulcers of Behcet's disease. Off-label use has been reported in diverse dermatological conditions, including aphthous stomatitis, chronic actinic dermatitis, atopic dermatitis, cutaneous sarcoidosis, hidradenitis suppurativa, lichen planus, and discoid lupus erythematosus. Roflumilast is a PDE4 inhibitor that was approved by the FDA and the EMA as an oral treatment of chronic obstructive pulmonary disease. Since patent expiration, several generic formulations of oral roflumilast have become available, and various studies have documented its off-label use in psoriasis and other dermatological conditions such as hidradenitis suppurativa, recurrent oral aphthosis, nummular eczema, lichen planus, and Behçet's disease. Topical roflumilast has received FDA approval for treatment of plaque psoriasis and seborrheic dermatitis. The favorable safety profile encourages its long-term use as an alternative to corticosteroids, addressing the chronic nature of many dermatological conditions. New oral PDE4 inhibitors are being developed, such as orismilast (LEO-32731), mufemilast (Hemay005), difamilast (OPA-15406) or lotamilast (E6005/RVT-501), among others. This narrative review provides a comprehensive synthesis of the pharmacology, clinical efficacy, safety profile, and practical considerations regarding the oral and topical use of PDE4 inhibitors in dermatology. [ABSTRACT FROM AUTHOR]

Objectives: To review the clinical features, treatment, and prognosis of coronary involvement in Behcet's syndrome in children. Methods: Retrospectively analyzed medical records of BS patients admitted to our institution from 2012 to 2024. Results: Six children with BS with coronary involvement were admitted, including 2 males and 4 females. The mean age was 5.7 years and the mean course was 17.2 months. All of them had coronary dilatation, including 1 case with aneurysmal dilatation, 2 cases with bilateral coronary dilatation, 4 cases with unilateral dilatation, and 4 cases with valvular disease. No symptom for coronary artery dilation and all of coronary dilatation were detected by echocardiography. Manifestations of extracardiac vascular involvement included arteriovenous wall thickness and thrombosis. Five cases had only arterial involvement and one case had both artery and vein involvement. Pulmonary artery was involved in 1 case. All of them were treated with glucocorticoid and immunosuppressant, 1 child was treated with TNF-α, and 1 received aortic valvuloplasty due to a large number of aortic regurgitations. The patient with coronary aneurysm died suddenly after giving up treatment, and the other 4 cases were all improved to varying degrees. Conclusion: Vascular involvement is an important factor affecting the prognosis of Behcet's syndrome. Patients with coronary involvement are occultic and difficult to diagnose. Both arteries and veins can be involved, and some patients have severe symptoms. Clinicians should attach great importance to early vascular screening, especially coronary artery. Early combined medication may help improve the prognosis. Key Points • Vascular involvement is an important factor affecting the prognosis of Behcet's syndrome. • Coronary involvement in BS is rare in children, which is predominantly coronary dilatation. • The onset of BS with coronary involvement is insidious and easy to be missed, so early coronary artery screening in children with BS should be emphasized. • Early combined medication may help improve the prognosis of BS with coronary involvement. [ABSTRACT FROM AUTHOR]

Behçet's disease is a chronic inflammatory condition that affects multiple organs and systems. It is characterized by recurrent oral and genital ulcers. A previous study reported that the IL-17A inhibitor secukinumab can improve the skin and mucosal manifestations in patients with refractory Behçet's disease. Additionally, secukinumab has been shown to effectively improve neurological symptoms when administered for Behçet's disease. However, we report a case where Behçet's disease occurred after the treatment of psoriasis with the IL-17A inhibitor ixekizumab. To summarize its clinical characteristics and treatment experience, we consulted relevant domestic and international literature and conducted a literature review. We concluded that anti-IL-17A treatment may lead to the development of Behçet's disease. The reported cases are more likely to occur in middle-aged men with varying onset times. The main manifestations include oral and vulvar mucosal ulcers. Furthermore, the gut microbiota may be involved in paradoxical Behçet's disease. [ABSTRACT FROM AUTHOR]

Copyright of Pamukkale Medical Journal is the property of Pamukkale Journal of Medicine and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background: When Behçet's disease is complicated with gastrointestinal ulcers, it is referred to as intestinal Behçet's disease (BD). Clinically uncommon, this condition can involve the entire gastrointestinal tract, often presenting diagnostic challenges in differentiation from Crohn's disease. Methods: In this case, atypical BD was diagnosed through endoscopic examination, whereas latent tuberculosis infection (LBTI) was confirmed via T-SPOT and PPD tests. Results: Methylprednisolone was administered during acute flare-ups to promote rapid ulcer healing. Adalimumab, meanwhile, provided swift treatment for intestinal BD and helped maintain long-term remission in affected patients. Additionally, isoniazid and rifampicin were used for the treatment of LBTI. Conclusions: This case demonstrates the complex and variable clinical course of BD, characterized by highly atypical presentations. As symptoms continue to develop and worsen over time, TNF-α inhibitors play a pivotal role in achieving long-term remission during treatment. [ABSTRACT FROM AUTHOR]

Copyright of Current Retina Journal / Güncel Retina Dergisi is the property of Anadolu Kitabevi Basim Yayim Medikal Turizm Kirtasiye Tic. Ltd. Sti. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Behçet's disease is an autoinflammatory disorder characterized by relapsing and remitting vasculitis that can manifest in various forms, including gastrointestinal Behçet's disease (GIBD). Its complications (e.g., intestinal perforation) are among the primary causes of morbidity and mortality. GIBD pathogenesis involves the enhanced production of certain cytokines, e.g., tumor necrosis factor α and interleukin-6 (IL-6), which could serve as a target for potential therapies. This review provides an overview of GIBD, including the diagnosis and immunopathogenesis as it is currently understood, and evaluates the emerging role of the inhibition of IL-6 (classic and trans-signaling) as an alternative treatment option for patients with GIBD. Given the current paucity of data, we reflected on the potential of IL-6 inhibitors such as tocilizumab and olamkicept based on immunopathogenic considerations and available clinical data in patients with inflammatory bowel disease (IBD), in whom clinical response or remission was induced. The selective inhibition of IL-6 trans-signaling may bring new impetus to the development of this drug class, particularly regarding safety. Still, the benefits of IL-6 inhibitors for patients with GIBD need to be evaluated in appropriate proof-of-concept studies. The clinical outcomes of IL-6 inhibitors in IBD are promising and may suggest their potential relevance in GIBD. [ABSTRACT FROM AUTHOR]

Introduction: Depression and anxiety are among the most prevalent mental health conditions in Brazil. Both are associated with poor quality of life (HRQoL) and challenges in disease management for chronic illnesses, including Behçet's disease (BD). This study aimed to evaluate depression, anxiety, and HRQoL in BD patients from a non-endemic area. Research design and methods: This case-control study included adult BD patients from Brazilian tertiary center and healthy controls (HC). All patients fulfilled the ISG and ICBD diagnostic criteria. Depression, anxiety and quality of life were assessed using BDI, HADS, SF-36, and physical capacity with the HAQ. Results: We enrolled 58 BD patients (60% female, mean age 46.1) and 96 HC (74% female, mean age 44). High rates of depression and anxiety were observed in BD patients, correlating with disease activity, younger age, absence of a partner, shorter disease duration, and lower income. BD patients showed significant HRQoL restrictions, particularly in physical and emotional roles, compared to HC. Longer disease duration was correlated with better HRQoL. Conclusion: High rates of depression and anxiety were observed in BD patients, negatively impacting HRQoL, particularly in those with higher disease activity. Further study and clinical attention are warranted to enhance patient care and outcomes. [ABSTRACT FROM AUTHOR]

Background: Patients with Behçet's disease (BD) who exhibit all of the physical and psychological symptoms and signs are likely to experience difficulties with daily living activities related to BD. The current study aimed to assess several factors that may be related to functional disability among BD patients studying these factors and targeting them to increase physical and mental activity as well as functional ability in the future. Results: Seventy-four BD patients were included in our study and they were 51 males and 23 females. BD patients had significantly higher Hospital Anxiety Depression Scale component for depression, and for anxiety, and Multidimensional Assessment of Fatigue scores than the healthy controls (P = 0.005, P < 0.001, and P < 0.001 respectively). A positive correlation was found significantly between the Modified Health Assessment Questionnaire and age (r = 0.2, P = 0.03), the duration of disease (r = 0.3, P = 0.01), disease activity (P = 0.008), and among patients with vascular (P = 0.02) and neurological affection (P = 0.002). Also, there was a highly significant positive correlation with anxiety, depression, and fatigue (P < 0.001). Conclusion: In BD patients, realizing the relationships between increased disability and aging, female sex, long disease duration, vascular and neurological involvement, different indices of activity, fatigue, anxiety, and depression was detected. Disease activity is one of the most important factors causing disability that should be controlled. Screening for depression, anxiety, and fatigue plus proper treatment is highly valued in promoting patient's coping with the disease. [ABSTRACT FROM AUTHOR]

Objectives: Current guidelines recommend systematic screening for rheumatic diseases (RDs), including antiphospholipid syndrome (APS), in patients with recurrent pregnancy loss (RPL). However, these recommendations are based on limited evidence, as data on the prevalence of RD in this specific population remain scarce. In particular, the impact of the recent update to the ACR/EULAR classification criteria for APS on the prevalence of RD among RPL patients has yet to be clarified. To address these gaps, this study aims to (i) assess the impact of the 2023 ACR/EULAR APS classification criteria in patients with recurrent pregnancy loss (RPL); and (ii) analyze the prevalence of RD in these patients. Methods: We conducted a retrospective cohort study at Rennes University Hospital. From January 2010 to December 2021, all patients referred to the Clinical Immunology Department for RPL were included. Patients were eligible if they had undergone a full RPL evaluation, according to guidelines. Results: We included 165 women with RPL. APS according to the Sydney criteria was found in 24 (14.5%) patients. No significant differences in obstetric history or clinical signs were observed between APS-positive and APS-negative individuals. Only two patients fulfilled the updated 2023 APS criteria, resulting in 163 (98.8%) patients being classified as having unexplained recurrent pregnancy loss (uRPL). Among them, 108 had a new pregnancy following uRPL, resulting in 87 (81%) live births and 21 (19%) recurrent miscarriages. We did not identify any prognostic factor associated with subsequent pregnancy outcomes, including the patients' antiphospholipid biological profile. We found a prevalence of non-APS RD of only 2.4% in the study population, including systemic lupus erythematosus, rheumatoid arthritis, and Behçet's disease. Conclusions: APS was identified in 14.5% of the patients based on the former Sydney criteria and 1.2% according to the revised criteria. The lack of clinical differences between APS and non-APS patients aligns with previously reported limitations of the Sydney criteria in accurately identifying aPLA-related RPL. According to the rarity of APS as per the updated criteria, future large collaborative trials will be needed to further characterize APS-related RPL patients and to determine the best treatment strategy for future pregnancies. [ABSTRACT FROM AUTHOR]

Background: Neurologic manifestations in the systemic autoimmune rheumatic diseases (SARDs) are protean. They add to the disease burden and could contribute to mortality. Increasing awareness about the neuro-rheumatologic syndromes might help with early diagnosis and effective therapy. Our aim is to survey the clinical and imaging patterns of neurological involvement in Egyptian patients with SARDs. Results: Neurological involvement is common in Behçet's disease (BD) (12.7%) and systemic lupus erythematosus (SLE) (6.4%) patients compared with other SARDs. Compared with SLE, neurological involvement in BD tends to develop at an older age (31 ± 7.1 versus 28.3 ± 9.6 years = 0.022) with a greater progression risk (13.8% versus 2.6%, P = 0.003). A higher proportion of SLE patients had abnormal neuroimaging without neurological symptoms (15.7% versus 4.3%, P = 0.026, OR = 4.9, 95%CI 1.1–22.4). SLE patients had a higher frequency of seizures (31.3% versus 6.4%, P < 0.001, OR = 6.7, 95%CI 2.7–16.7) and benign intracranial hypertension (9.6% versus 1.1%, P = 0.009, OR = 9.8, 95%CI 1.2–77.7) but a lower prevalence of quadriplegia due to brain insult (1.7% versus 3.2%, P = 0.045, OR = 0.2, 95%CI 0.04–0.9), dural sinus thrombosis (13% versus 33%, P = 0.001, OR = 0.3, 95%CI 0.2–0.6), brainstem syndrome (0.9% versus 6.4%, P = 0.047, OR = 0.1, 95%CI 0–1.1) and cranial neuropathies (9.6% versus 31.9%, P < 0.001, OR = 0.2, 95%CI 0.1–0.5). Concerning neuroimaging, brain atrophic changes were more common (27.4% versus 9.5%, P = 0.002, OR = 3.6, 95%CI 1.6–8.3) while thrombosis was less prevalent (36.3% versus 53.6%, P = 0.016, OR 0.5, 95%CI = 0.3–0.9) in lupus patients. The cerebral cortex was more commonly affected (20.4 versus 4.8%, P = 0.002, OR = 5.1, 95%CI 1.7–15.4) while dural sinuses (14.2% versus 40.5%, P < 0.001, OR = 0.2, 95%CI 0.1–0.5), basal ganglia (1.8% versus 10.7%, P = 0.010, OR = 0.2, 95%CI 0–0.7), diencephalon (0% versus 13.1%, P < 0.001) and brainstem (1.8% versus 22.6%, P < 0.001, OR = 0.1, 95%CI 0–0.3) were less frequently involved in SLE patients. Concerning other SARDs, cranial neuropathies were the most common neurological presentations. Abnormalities in neuroimaging did not correlate with the patients' clinical presentations. Conclusions: Neurological presentations associated with SARDs are protean. Neuroimaging abnormalities should be interpreted within the context of the clinical picture and the results of other investigations. Key points: • What is already known about this subject? Neurological involvement has been described in almost all types of systemic autoimmune rheumatic diseases (SARDs); it might contribute significantly to the disease burden, disease-related morbidity and mortality. Neurological affection might develop at any time along the course of SARDs and might even precede the onset of systemic manifestations. The spectrum of neurological manifestations in patients with SARDs is quite pleomorphic and varies regarding the frequency; etiology; pathology, vascular or parenchymal, as well as the distribution, peripheral, central or both. • What does this study add? The study demonstrated that patients with SARDs could present with protean neurological presentations with some are the most common while others are relatively characteristic. Abnormalities on neuroimaging do not necessarily correlate with the neurological presentation of the patient. Hence, neuroimaging abnormalities should be interpreted within the context of the clinical picture of the patient and the results of other investigations. • How might this impact on clinical practice? Certain syndromes or presentations are relatively unique to a specific SARD. Increasing the awareness of these specific neuro-rheumatologic syndromes might support earlier diagnosis and effective therapy; this is of a particular concern when neurological manifestations are the presenting features of SARDs. [ABSTRACT FROM AUTHOR]

ABSTRACT Aims Methods and results Conclusion Although certain autoimmune diseases (AIDs) have been associated with an increased rate of heart failure (HF), data on the long‐term rate of HF across the spectrum of AIDs are lacking. We investigated the long‐term rate of HF in individuals with a history of 28 different AIDs.Individuals diagnosed with an AID (2000–2021) were identified through Danish nationwide registries. Each patient with AID was matched with four individuals from the background population by age, sex, and year of inclusion. Multivariable Cox regression was used to compare the rate of HF between the AID and the background population, overall and according to individual AIDs. In total, 272 959 patients diagnosed with AID were matched with 1 091 836 individuals without AID (median age 55 years; 62% women; median follow‐up 7.9 years). The 10‐year cumulative incidence of HF was 5.2% for patients with AID and 3.5% for matched individuals. Patients with any AID had a higher associated rate of HF than matched individuals (hazard ratio [HR] 1.55, 95% confidence interval [CI] 1.52–1.59). Patients with each of the AIDs had a higher associated rate of incident HF compared with matched individuals from the background population, although the association was not statistically significant for Reiter's and Behcet's disease. The highest relative rates were observed in patients with systemic sclerosis (HR 3.31, 95% CI 2.63–4.16) and Addison's disease (HR 3.03, 95% CI 2.35–3.91).Patients with AID, irrespective of the type, had a higher associated rate of HF compared to the background population. Further research is needed to clarify whether screening for cardiovascular risk is beneficial. [ABSTRACT FROM AUTHOR]

Objectives: The study aimed to evaluate the frequency of diastolic dysfunction (DD) in patients with Behçet's disease (BD) and its relationship with clinical manifestations. Patients and methods: Seventy patients (35 females, 35 males; mean age: 39.3±11.0 years; range, 21 to 61 years) with BD and 50 healthy controls (27 females, 23 males; mean age: 38.8±10.0 years; range, 23 to 62 years) were included in the case-control study between October 2023 and December 2023. Age, sex, smoking status, disease duration, mucocutaneous involvement, other organ involvement, treatment received of the patients were recorded. The E/A ratio (the ratio of early diastolic wave to the atrial wave) and the E/E' ratio (the ratio of early transmitral filling velocity to early diastolic mitral annular velocity), which are markers of DD, were measured by transthoracic echocardiography. Results: Diastolic dysfunction was observed in 27.1% (n=19) of BD patients and 8% (n=4) of healthy controls (p=0.017). Male dominance was observed in patients with major organ involvement (MOI) compared to patients with isolated mucocutaneous involvement (p=0.001). While MOI was observed at a rate of 65.7%, ocular involvement, among the MOIs, constituted 52.9% of all BD patients. The percentage of patients with a mitral E/A ratio <1 was similar between the MOI and isolated mucocutaneous involvement groups. However, vascular involvement was observed in 47.4% of BD patients with a mitral E/A ratio <1 (p=0.034). In addition, it was observed that the group with a mitral E/A ratio <1 was older, had a longer disease duration, and had a higher erythrocyte sedimentation rate. Conclusion: Diastolic dysfunction is an early sign of cardiac involvement, and prolonged vascular involvement in BD increases the risk of DD. [ABSTRACT FROM AUTHOR]

Objectives: This study aimed to evaluate the performance of the Behçet's Syndrome Overall Damage Index (BODI) in an adult Egyptian cohort. Patients and methods: This longitudinal retrospective cohort study included 282 adult patients (233 males, 49 females; mean age: 35.3±8.7 years; range, 16 to 66 years) with Behçet's disease (BD) between January 1980 and December 2022. BODI was assessed regarding construct validity, sensitivity to change, and intra- and inter-rater reliability. The ability of BODI to discriminate between activity and damage was evaluated. Its performance in another vasculitis syndrome was assessed in 12 patients with antineutrophil cytoplasmic antibody-associated vasculitis. Results: BODI captured more damage items compared to the Vasculitis Damage Index (VDI). BODI scores were not correlated with disease activity and had poor performance in other vasculitides. BODI had a trend of progressive increment over time. It showed consistence when reassessed by the same rater and by different raters. Some damage items in the study cohort were lacking in BODI. Conclusion: BODI is more comprehensive compared to VDI. It shows good face, construct, and discriminant validity. It is sensitive to change and has good intra- and inter-rater reliability. Newer versions of BODI are recommended to increase score comprehensiveness. [ABSTRACT FROM AUTHOR]

Objectives: The purpose of this study was to evaluate Eustachian tube function in patients with Behçet's disease (BD). Patients and methods: Forty-six patients (22 males, 24 females; mean age: 42.1±11.3 years; range, 19 to 64 years) with BD and 46 (21 males, 25 females; mean age: 38.5±14.8 years; range, 19 to 63 years) age- and sex-matched audiologically healthy individuals were enrolled in this cross-sectional, case-control study between June 2023 and August 2023. Demographic and clinical characteristics of participants were recorded from electronic health records. All participants completed the Eustachian tube function test and the Eustachian tube dysfunction questionnaire 7 (ETDQ-7). Binary logistic regression analysis was employed to identify the factors that predict Eustachian tube dysfunction in patients with BD. Additionally, the association between disease duration and ETDQ-7 scores was evaluated using Spearman's rank correlation. Results: The median ETDQ-7 scores were significantly higher in patients with BD than in controls (10 (7-32) vs. 7 (7-9); p<0.001). The number of ears with Eustachian tube dysfunction was also significantly higher in the BD group than in the controls (n=22, 47.8% vs. n=7, 15.2%; p=0.007). Regression analysis did not reveal statistically significant factors that predicted Eustachian tube dysfunction. Moreover, no statistically significant correlation was observed between Eustachian tube dysfunction and disease duration (R=-0.067; p=0.525). Conclusion: This study showed that Eustachian tube dysfunction is more prevalent in patients with BD than in the healthy controls. However, clinical and demographic variables were not found to be associated with Eustachian tube dysfunction. [ABSTRACT FROM AUTHOR]

Behçet's syndrome is a variable vessel vasculitis characterized by a diverse range of clinical manifestations resulting from inflammation involving several organs and systems. While significant progress has been made in understanding the pathogenesis and treatment of Behçet's syndrome, challenges remain in achieving optimal disease control and preventing long-term complications. This review explores recent advances in the management of Behçet's syndrome, with a focus on emerging therapies and future directions. Apremilast, a phosphodiesterase-4 inhibitor, has shown promise in managing mucocutaneous manifestations, particularly oral ulcers. Tocilizumab, an interleukin (IL)-6 receptor inhibitor, has demonstrated efficacy in certain patient populations, especially those with ocular involvement. However, its use in vascular Behçet's syndrome requires careful consideration. Relapses of oral and genital ulcers can be challenging during tocilizumab treatment. Other emerging therapies, such as IL-17 inhibitors, including secukinumab and ixekizumab, IL-12/23 inhibitor ustekinumab, and Janus kinase (JAK) inhibitors, including tofacitinib and baricitinib, are being investigated for their potential to target specific inflammatory pathways. Future research directions include the development of novel therapeutic targets, better use of existing agents by identifying patient populations that would benefit from these, developing better instruments for disease assessment, and a treat-to-target approach in order to improve outcomes and quality of life for patients with Behçet's syndrome. [ABSTRACT FROM AUTHOR]

Background: The leading cause of blindness due to non-infectious uveitis is cystoid macular edema (CME). Behçet's disease (BD) is one of the most commonly conditions related to CME. Objectives: To compare the effectiveness and safety of adalimumab (ADA), infliximab (IFX) and certolizumab (CZP) in refractory CME due to BD. Methods: Multicenter study of BD-CME patients with no response to glucocorticoids (GCs) and at least one conventional immunosuppressive drug. At baseline, all patients presented CME, defined by OCT > 300 µ. The effectiveness of ADA, IFX and CZP was assessed over a 2-year period from baseline using the following ocular parameters: macular thickness (µm), visual acuity (BCVA), anterior chamber (AC) cells and vitritis. Mixed-effects regression models were applied. Results: a total of 50 patients (75 eyes) were studied (ADA = 25; IFX = 15 and CZP = 10). No significant differences in demographic parameters were found among the three groups. However, individuals in the CZP group had a significantly extended time from diagnosis to treatment onset (72 (36–120) months, p = 0.03) and had received a higher number of biological therapies (1.7 ± 1.1) compared to the ADA and IFX groups. Within the CZP group, ADA and IFX were previously administrated in seven patients. After 2 years of follow-up, a rapid and sustained reduction in macular thickness was noted in all three groups with no significant differences between them. Additionally, enhancements in BCVA, AC cells and vitritis were also observed. No serious adverse events were reported in the CZP group, although one isolated case of bacteremia was documented in the ADA group. ADA, IFX and CZP appear to be effective and safe treatments for refractory CME in BD. CZP seems to remain effective even in patients with an insufficient response to ADA and/or IFX. Conclusions: ADA, IFX and CZP appear to be effective and safe treatments for refractory CME in BD. CZP seems to remain effective even in patients with an insufficient response to ADA and/or IFX. [ABSTRACT FROM AUTHOR]

Introduction/Objective: There is currently no tool available to assess the severity of damage in uveitis due to Behçet's syndrome (BS). In this preliminary study, we developed a new grading system to evaluate ocular damage and assessed it in a prospective cohort. Methods: A specialist in BS uveitis (YO) developed a grading system for ocular damage with five grades based on the extent of damage in the posterior segment. YO trained a senior and general ophthalmologist with sample fundus images. BS patients who had undergone color fundus photography during their routine visits in attack-free periods were included in the study. The color fundus photos of this prospective cohort were evaluated blindly by YO and his trainees using the new grading tool. Inter and intra-observer agreement between the graders were assessed by Cohen's kappa analysis. The evaluation of YO was considered as the gold standard. Results: One hundred eighty-five eyes of 108 (29 F/79 M) patients with BS uveitis were graded for damage by two investigators. Their mean age was 38,58 years and their median ocular disease duration was 13 years. The gold standard and the two investigators exhibited substantial concordance with the ocular damage grading system. The inter- and intra-observer agreement were also almost perfect. Conclusion: The newly developed ocular damage grading system enables the standardization of damage severity in BS uveitis. It is imperative to conduct internal and external validations across diverse cohorts. Furthermore, future studies should investigate its correlation with other multimodal imaging methods such as fluorescein angiography and optical coherence tomography. [ABSTRACT FROM AUTHOR]

Trisomy 8 is a frequent chromosomal abnormality that occurs among patients with myelodysplastic syndrome (MDS). This abnormality is more common among MDS patients with concurrent Behcet's disease (BD) than among patients with MDS alone. This comorbidity is also associated with a higher prevalence of intestinal ulcers. In this case report, we describe a 45-year-old woman who was diagnosed with BD at age 35. Eight years later, her Behcet's disease relapsed with trisomy 8-positive MDS. After the disease was controlled by azacitidine, the patient received allogeneic hematopoietic stem cell transplantation (allo-HSCT), which involved the use of both peripheral blood stem cells and bone marrow stem cells. Although the patient experienced grave oral mucositis and chronic intestinal graft-versus-host disease (GVHD), remission of both MDS and BD was maintained. This case highlights the potential efficacy of allo-HSCT in treating patients with refractory autoimmune diseases complicated by MDS, particularly patients with trisomy 8 abnormalities. We also discuss the immunological implications and suggest allo-HSCT as a viable curative option for patients with BD and MDS. [ABSTRACT FROM AUTHOR]

Tuberculosis (TB) is an infectious disease caused by Mycobacterium tuberculosis bacteria, which is more prevalent among immunocompromised individuals. According to the distribution of affected organs, this infection can be categorized as either pulmonary or extrapulmonary TB. Immunodeficiency states resulting from rheumatological disorders and the use of immunosuppressive medications, such as in Behçet's disease (BD), are potential predisposing factors for TB, particularly in cases involving multiple organs. These situations can introduce challenges in both the diagnosis and treatment of patients. We describe a 43‐year‐old man with a history of BD who presented with symptoms of weight loss, abdominal pain, and shortness of breath. His chest X‐ray revealed cavities and calcifications, while an abdominal X‐ray demonstrated signs of intestinal obstruction and adhesions. Subsequent TB diagnosis led to a 6‐month course of a TB treatment regimen. Despite treatment initiation, the patient developed a brain abscess 1 year later, necessitating surgical intervention. Following the procedure, he received another 1‐year course of a TB treatment regimen and experienced full recovery without any complications during a 2‐year follow‐up period. Notably, the patient recently received a Sinopharm COVID‐19 vaccine and subsequently developed seizures that are currently being managed with anticonvulsant therapy. This case report emphasizes the significance of including pulmonary TB in complex medical cases, especially in individuals with autoimmune diseases. Early diagnosis and treatment are crucial for improving outcomes and reducing the risk of complications. Furthermore, it highlights the possible correlation between TB and BD, along with the potential adverse reactions to COVID‐19 vaccines in this population, which necessitate special consideration by healthcare professionals. [ABSTRACT FROM AUTHOR]

This case highlights the importance of considering Neuro‐Behçet's disease (NBD) in the differential diagnosis of multiple sclerosis (MS), particularly in patients presenting with neurological manifestations, abnormal magnetic resonance imaging (MRI) findings, and systemic symptoms consistent with Behçet's disease (BD). [ABSTRACT FROM AUTHOR]

The article discusses the importance of extended vascular screening in asymptomatic Behçet's disease patients with previously diagnosed vascular involvement or unexplained inflammatory response. It highlights the various manifestations of Behçet's disease, such as mucocutaneous symptoms, ocular, vascular, gastrointestinal, and neurological manifestations. The text emphasizes the need for early detection and monitoring of vascular disease in Behçet's patients, especially in high-risk populations, to prevent severe complications like Budd–Chiari syndrome and arterial thrombosis/aneurysms. The authors recommend close follow-up and screening for suspected symptoms in young male patients with a severe mucocutaneous course, as well as screening for other vascular regions in patients with a history of vascular involvement. [Extracted from the article]

Objective: This study aimed to investigate the efficacy and safety of a triple therapy consisting of colchicine, thalidomide and total glucosides of paeony (TGP) in Behcet's disease (BD) patients with mucocutaneous involvement. Methods: Totally 355 newly diagnosed BD patients with mucocutaneous involvement were recruited, who received dexamethasone and colchicine for the first 2 weeks, then they were categorized into "sustained triple‐therapy (ST)" (n = 231) and "colchicine to triple‐therapy (CT)" (n = 124) groups respectively: for ST group, patients received colchicine, thalidomide plus TGP from Month (M)0.5 to M12; for CT group, patients received colchicine from M0.5 to M2, then switched to colchicine, thalidomide plus TGP from M3 to M12. Results: The percentages of oral ulceration (at M1, M2) and genital ulceration (at M1) were lower in ST group compared to CT group, whereas there was no difference of other clinical manifestations (including uveitis, erythema nodosum, thrombosis, arterial involvement or nervous system involvement) at each time point between the two groups. For biochemical indexes, ESR was higher at M1 but rapidly reduced at M2 in ST group compared to CT group, while CRP level was similar at all time points between the two groups. For side effects, occurrences of drug‐related cytopenia and diarrhea were increased, in ST group compared to CT group. Conclusions: A triple therapy consisting of colchicine, thalidomide and TGP is more effective and equally tolerated compared to colchicine alone in treating BD patients with mucocutaneous involvement. [ABSTRACT FROM AUTHOR]

Pulmonary artery aneurysmal rupture is a rare cause of massive hemoptysis. When the suspected origin of bleeding is the pulmonary artery, comprehensive evaluation is necessary to determine aetiology and guide appropriate management. Behçet's disease and Hughes–Stovin Syndrome (HSS) are important differentials to consider after infections and malignancy have been ruled out. Pulmonary artery aneurysms with aneurysmal wall enhancement and 'in‐situ' thrombus should prompt the suspicion of HSS. Early diagnosis and treatment with immunosuppressants and endovascular interventions in selected patients may improve the prognosis and prevent episodes of fatal hemoptysis. [ABSTRACT FROM AUTHOR]

Objective: To assess the proportion of postoperative complications of various surgeries in patients with Behçet's disease (BD) and compare the risk of surgical complications between BD patients and controls. Methods: We analyzed 389 BD patients who underwent surgeries at Seoul National University Hospital between January 2003 and December 2019. Controls were 1:1 matched with BD patients based on age at surgery, sex, and type of surgery. Generalized estimating equation analyses were performed to compare the postoperative complications between BD patients and controls. Results: Among 632 surgeries in BD patients, 36 (5.7%) surgical site complications, including wound dehiscence (38.9%), bleeding (13.9%), infections (8.3%), anastomotic dehiscence (22.2%), and stricture (5.6%), occurred after median 12 days (IQR 7.8–22.0). Surgical site complications developed frequently after cardiac (33.3%) and vascular (22.2%) surgeries and rarely occurred after ophthalmic (1.5%) surgeries. Seventeen (2.7%) cases required reoperation within median 15 days (IQR 7.0–43.0). Four patients died within median 21 days (IQR 8.3–41.3 days). After adjustment for confounders, BD patients exhibited a significantly higher risk of surgical site complications (OR 3.4, 95% CI 1.4–8.0) and reoperation (OR 5.2, 95% CI 1.2–22.8) after cardiovascular surgery than controls. However, the risks of surgical site complications and reoperation after other types of surgery were similar in both groups. Conclusions: The risk of postoperative complications varies according to the type of surgeries among BD patients. While cardiovascular surgeries result in higher perioperative complications in BD patients, ophthalmic surgeries show a safety profile similar to those in non-BD patients. Key Points • Patients with Behçet's disease exhibited an increased risk of surgical site complications and subsequent reoperations after cardiovascular surgery than controls. • The safety profile of ophthalmic surgery was comparable between patients with Behçet's disease and controls. • In patients with Behçet's disease, the risk of postoperative complications varies according to the type of surgery. [ABSTRACT FROM AUTHOR]

Objective Several monogenic autoinflammatory disorders and primary immunodeficiencies can present early in life with features that may be mistaken for Behçet's disease (BD). We aimed to develop a genetic analysis workflow to identify rare monogenic BD-like diseases and establish the contribution of HLA haplotype in a cohort of patients from the UK. Methods Patients with clinically suspected BD were recruited from four BD specialist care centres in the UK. All participants underwent whole-exome sequencing (WES), and genetic analysis thereafter by (i) examining genes known to cause monogenic immunodeficiency, autoinflammation or vasculitis by virtual panel application; (ii) scrutiny of variants prioritized by Exomiser using Human Phenotype Ontology (HPO); (iii) identification of copy number variants using ExomeDepth; and (iv) HLA-typing using OptiType. Results Thirty-one patients were recruited: median age 15 (4–52), and median disease onset age 5 (0–20). Nine/31 (29%) patients had monogenic disease mimicking BD: five cases of Haploinsufficiency of A20 with novel TNFAIP3 variants (p.T76I, p. M112Tfs*8, p. S548Dfs*128, p. C657Vfs*14, p. E661Nfs*36); one case of ISG15 deficiency with a novel nonsense variant (ISG15 : p.Q16X) and 1p36.33 microdeletion; one case of common variable immune deficiency (TNFRSF13B : p.A181E); and two cases of TNF receptor-associated periodic syndrome (TNFRSF1A : p.R92Q). Of the remaining 22 patients, eight (36%) were HLA-B*51 positive. Conclusion We describe a novel genetic workflow for BD, which can efficiently detect known and potentially novel monogenic forms of BD, whilst additionally providing HLA-typing. Our results highlight the importance of genetic testing before BD diagnosis, as this has an impact on choice of therapy, prognosis and genetic counselling. [ABSTRACT FROM AUTHOR]

Objectives To validate the PEDiatric Behçet's Disease classification criteria (PEDBD) with an evidence-based approach. Methods A total of 210 pediatric patients [70 Behçet's disease (BD), 40 periodic fever, aphthous stomatitis, pharyngitis, adenitis, 35 familial Mediterranean fever, 26 hyper-IgD syndrome, 22 TNF-receptor associated periodic fever syndrome, 17 undefined recurrent fevers] were randomly selected from the Eurofever Registry. A set of 11 experienced clinicians/researchers blinded to the original diagnosis evaluated the patients. Using the table consensus as gold standard (agreement ≥ 80%), the PEDBD, ISG and ICBD criteria were applied to BD patients and to confounding diseases with other autoinflammatory conditions in order to define their sensitivity, specificity and accuracy. Results At the end of the third round, a consensus was reached in 139/210 patients (66.2%). The patients with a consensus ≥80% were classified as confirmed BD (n  = 24), and those with an agreement of 60–79% as probable BD (n  = 10). When comparing these patients with the confounding diseases group, an older age at disease onset, the presence of oral and genital ulcers, skin papulo-pustular lesions, a positive pathergy test and posterior uveitis were BD distinctive elements. The ISG, ICBD and PEDBD criteria were applied to confirmed BD and to the confounding disease group, showing a sensitivity of 0.50, 0.79 and 0.58, a specificity of 1.00, 0.97 and 0.99, and an accuracy of 0.91, 0.94 and 0.92, respectively. Conclusions The PEDBD criteria were very specific, while the ICBD were more sensitive. The complexity of childhood BD suggests larger prospective international cohorts to further evaluate the performance of the criteria. [ABSTRACT FROM AUTHOR]

Background: The course of late‐onset Behçet's disease (LBD) is unknown. This study aimed to determine the characteristics and systemic involvement of LBD. Methods: In this retrospective, cross‐sectional, and comparative study, 700 patient files from 4100 patients diagnosed with Behçet's disease (BD) were selected and divided into three groups: LBD, adult BD (ABD), and juvenile BD (JBD). The age limits for LBD and JBD were determined to be 42 and 16, respectively. LBD patients were compared to ABD and JBD patients in terms of demographics, systemic involvement, and disease duration. Results: The LBD rate among BD patients was 4.7% (183/4,100). The time from initial symptom occurrence to the age at which the BD criteria were met was longer in LBD than in ABD or JBD. Except for genital ulcers, the frequencies of involvement in LBD were similar to those in ABD and JBD. The frequency of family history was significantly lower in LBD (12%) than in ABD (15.9%) or JBD (18.2%). The time from the initial symptom to oral aphthae, genital ulcers, and eye involvement was longer in LBD than in ABD or JBD. Furthermore, erythema nodosum was observed after a longer duration in LBD than in ABD. Conclusion: Considering that involvement occurs much later in LBD and there are no differences in the frequencies of involvement except for genital ulcers, LBD patients should be followed up as closely as ABD and JBD patients. [ABSTRACT FROM AUTHOR]

Purpose: To describe the treatment outcomes of adalimumab in noninfectious pediatric uveitis. Methods: The electronic medical records of children with noninfectious uveitis were reviewed retrospectively. The visual improvement and ocular inflammation were assessed according to the Standardization of Uveitis Nomenclature criteria before and after treatment with adalimumab. Results: Twenty-nine eyes of 16 patients were included. The mean age of children was 9.4 ± 3.8 years. There were eight (50%) males and eight (50%) females. Juvenile idiopathic arthritis was the most common cause of uveitis in these children (N = 10, 62.5%), followed by Behçet's disease (N = 3, 18.75%) and Vogt–Koyanagi–Harada disease (N = 2, 12.5%). One child (6.25%) was diagnosed with Crohn's disease. Nine (56.3%) children were previously treated with methotrexate, one each with mycophenolate mofetil (MMF) (6.3%), azathioprine (6.3%), and tofacitinib (6.3%), and one child received MMF followed by azathioprine and cyclosporine before shifting to adalimumab. Three (18.8%) children did not receive prior immunosuppressive therapy. The average number of adalimumab injections given was 20.3 ± 12.0 at an interval of 2 weeks. The mean follow-up duration was 22.2 ± 16.6 months. Disease inactivity was achieved in 28 (96.6%) eyes at last visit (P < 0.001). The best-corrected visual acuity (BCVA) improved from 0.48 ± 0.6 logMAR (logarithm of minimum of resolution) units at baseline to 0.20 ± 0.2 logMAR units at last visit (P = 0.018). Disease remission was achieved in 23 (79.3%) eyes at the third month (P = 0.001). Conclusion: Adalimumab is effective in the treatment of pediatric noninfectious uveitis by achieving disease inactivity, reducing the recurrence rate and improving BCVA. The drug is safe and well tolerated. [ABSTRACT FROM AUTHOR]

Introduction: Behçet's syndrome (BS) is a chronic multisystem disease that mainly occurs along the ancient Silk Road. Uveitis is a major manifestation of BS with potentially a high visual morbidity. Early diagnosis of BS uveitis (BSU) based on characteristic ocular findings can be helpful in appropriate management of the patients. Areas covered: A comprehensive literature review was conducted in PubMed database. New advances in the diagnosis and management of BSU were reviewed. Expert opinion: The diagnosis of BSU is based on a combination of clinical findings. New sets of diagnostic/classification criteria have been developed including the International Criteria for Behçet Disease and the Standardization of Uveitis Nomenclature (SUN) working group criteria. An algorithm for the diagnosis of BSU based on characteristic ocular findings has been recently proposed to help ophthalmologists in predicting BS diagnosis in patients presenting with isolated uveitis. Treatment of BSU is currently based on the European Alliance of Associations for Rheumatology (EULAR) and the French recommendations. Treatment of anterior uveitis is based on topical corticosteroids with mydriatics unless there are high risk factors for poor visual outcomes. Posterior segment involvement requires the combination of systemic corticosteroids, and immunosuppressive or immunomodulating agents. Anti TNFα or IFN-α2a should be considered in non-responsive patients or in those with acute severe inflammatory attack. [ABSTRACT FROM AUTHOR]