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Background: Non‐Hodgkin lymphoma (NHL) is one of the most common haematologic malignancies in the world. Despite substantial efforts to identify causes and risk factors for NHL, its aetiology is largely unclear. Autoimmune diseases have long been considered potential risk factors for NHL. We carried out Mendelian randomisation (MR) analyses to examine whether genetically predicted susceptibility to ten autoimmune diseases (Behçet's disease, coeliac disease, dermatitis herpetiformis, lupus, psoriasis, rheumatoid arthritis, sarcoidosis, Sjögren's syndrome, systemic sclerosis, and type 1 diabetes) is associated with risk of NHL. Methods: Two‐sample MR was performed using publicly available summary statistics from cohorts of European ancestry. For NHL and four NHL subtypes, we used data from UK Biobank, Kaiser Permanente cohorts, and FinnGen studies. Results: Negative associations between type 1 diabetes and sarcoidosis and the risk of NHL were observed (odds ratio [OR] 0.95, 95% confidence interval [CI]: 0.92–0.98, p = 5 × 10−3, and OR 0.92, 95% CI: 0.85–0.99, p = 2.8 × 10−2, respectively). These findings were supported by the sensitivity analyses accounting for potential pleiotropy and weak instrument bias. No significant associations were found between the other eight autoimmune diseases and NHL risk. Conclusion: These findings suggest that genetically predicted susceptibility to type 1 diabetes, and to some extent sarcoidosis, might reduce the risk of NHL. However, future studies with different datasets, approaches, and populations are warranted to further examine the potential associations between these autoimmune diseases and the risk of NHL. [ABSTRACT FROM AUTHOR]

Vasculitis in children represents a diverse group of diseases characterized by inflammation of blood vessels, which can lead to significant morbidity if not promptly recognized and managed. This review explores the epidemiology, pathophysiology, classification, and management of key pediatric vasculitides. Classification based on vessel size aids in diagnosis and treatment. Understanding these conditions” clinical features and therapeutic options is critical for improving pediatric patient outcomes and preventing long-term complications. Continued research is essential for refining treatment strategies and enhancing patient care. [ABSTRACT FROM AUTHOR]

Behcet's disease (BD) is a multisystem disorder prevalent along the historic Silk Road, with Behcet's uveitis (BU) representing a significant complication contributing to disability. Various studies have linked different HLA alleles with BD across diverse populations. In this study, we investigated the association between HLA-B51:01/x and HLA-B27/x genotypes with Behcet's uveitis in 50 unrelated Iranian patients diagnosed with Behcet's uveitis, comparing them to a control group of 70 healthy individuals. Our analysis aimed to determine the susceptibility conferred by these alleles and assess their clinical relevance. Our findings indicate a notable susceptibility conferred by the HLA-B51:01/x genotype for Behcet's uveitis (P = 0.0001). Conversely, the B27/x genotype did not demonstrate significant associations with Behcet's uveitis. Furthermore, we employed prevalence-corrected positive predictive value (PcPPV) calculations to gauge the clinical utility of testing for these alleles within the Iranian Behcet's uveitis patient population. The PcPPV for B27/x genotype testing was determined to be 0.05%, while the PcPPV for B51:01/x genotype testing in the same population was 0.065%. These results suggest that carriers of the B*51:01 allele, when presenting with clinical symptoms, exhibit a heightened risk for Behcet's uveitis compared to the general population. Individuals carrying the B51:01 allele, when symptomatic, face an elevated Behcet's uveitis risk. This insight aids in targeted clinical assessments for at-risk populations. [ABSTRACT FROM AUTHOR]

Objectives: Chronic diseases impact people's quality of life (QoL). Behçet's disease (BD) is a multisystemic chronic disease characterized by vasculitis of various vessels. We aimed to assess QoL in paediatric BD (PEDBD) patients and their parents. Methods: We conducted a cross-sectional study from June to December 2022, including PEDBD patients meeting classification criteria. We recorded clinical characteristics and assessed QoL using PedsQL for patients and WHOQOL-Bref for parents. Results: A study of 38 patients (60.5% girls, 39.5% boys), median age 15.5 years (range 10–17), with oral aphthae and various mucocutaneous symptoms: genital ulcers (78.9%), pseudofolliculitis (76.3%), erythema nodosum (23.6%), and some with uveitis (13.1%), vascular (13.1%), neurological (10.5%), and gastrointestinal (5.2%) involvement. All were in remission under treatment. Median PedsQL scores: total 74.5 (range 40–94.8), physical health 76.5 (43–100), psychosocial health 75 (25–92); 14 patients scored below cutoff. Girls had lower physical health scores (P = .004), and disease duration correlated negatively with PedsQL score (r = -0.648, P = .001). Median WHOQOL score for parents: 50 (25–100), with 20 scoring below cutoff. Conclusion: One-third of PEDBD children and over half of their parents had low QoL. Disease duration and gender, as with many chronic illnesses, correlated with QoL in PEDBD. [ABSTRACT FROM AUTHOR]

Objectives: Specific human leukocyte antigen (HLA) haplotypes are associated with Behçet's disease. Because the effects of HLA-A26 and its combination with HLA-B51 on organ involvement in Behçet's disease have not been well demonstrated, we aimed to examine them. Methods: This multicentre, cross-sectional, observational study enrolled patients with Behçet's disease who visited Kyoto University Hospital between 2018 and 2021 or Kurashiki Central Hospital between 2006 and 2016 (n = 200). Disease severity was evaluated using the Krause score. Results: Uveitis and gastrointestinal involvement were observed in 95/196 and 57/167 patients, respectively. The HLA alleles identified were HLA-B51 (n = 52/106), HLA-A26 (n = 25/88), and HLA-B51 and HLA-A26 (n = 6/88). In patients harbouring HLA-B51, the presence of HLA-A26 was associated with higher frequencies of uveitis (P = .03) and coexistence of uveitis and gastrointestinal involvement (P = .002) and higher Krause scores (P = .02). Furthermore, the presence of HLA-A26 was associated with a higher frequency of uveitis in patients with gastrointestinal involvement (P = .001) and gastrointestinal involvement in patients with uveitis (P = .001). Conclusions: Since specific HLA haplotypes and their combinations are associated with organ involvement, both HLA-A and HLA-B haplotypes should be confirmed when screening for affected organs. [ABSTRACT FROM AUTHOR]

The British Journal of Dermatology published a living guideline in 2024 for managing Behçets disease/syndrome, developed by the British Association of Dermatologists and the British Society for Rheumatology. The guideline includes evidence-based recommendations for adults, children, and young people with a focus on personalized care and shared decision-making. It emphasizes a holistic, patient-centered approach to optimize disease management and improve quality of life, highlighting the importance of multidisciplinary team care, tailored treatment strategies, and psychological support for patients. The document also calls for further research, including genetic studies and clinical trials, to enhance understanding and treatment outcomes for individuals with Behçets. [Extracted from the article]

Single nucleotide polymorphisms (SNPs) of the protein tyrosine phosphatase non-receptor type 2 (PTPN2) gene have been documented to be linked with several autoimmune disorders including Behçet's disease (BD). PTPN2 SNPs rs2542151 and rs7234029 have been assessed using real-time PCR in 96 BD patients and 50 controls matched by age and gender. Patients were categorized into groups according to the disease phenotypes and severity. A total of 94.8% of patients were males. The patients' mean age at onset was 26.1 ± 8 years. The median (IQR) disease duration was 8.5(4–13) years. No difference was observed between the patients and controls concerning the frequency of the two SNPs' different genotypes, models, and alleles. Moreover, neither disease phenotypes nor severity were associated with rs2542151 or rs7234029 SNPs. PTPN2 rs2542151 and rs7234029 SNPs do not seem to have associations with BD occurrence, phenotypes, or severity in the Egyptian patients. Key Points • PTPN2 rs2542151 and rs7234029 SNPs do not seem to have associations with BD occurrence, phenotypes, or severity in the Egyptian patients. • Further studies involving a larger sample size with variable clinical diversity are recommended to verify the results. [ABSTRACT FROM AUTHOR]

Enthesitis is a characteristic manifestation of spondyloarthropathy (SpA). Historically, Behçet's syndrome (BS) was classified within SpA. Although they are now classified separately, the association between BS and SpA remains controversial. The concept of MHC-I (major histocompatibility complex class I)-opathy has been proposed based on the overlap in immunopathological mechanisms among diseases associated with human leukocyte antigen (HLA) class I. Enthesitis is a frequent complication in patients with BS who also have acne and arthritis. However, information regarding enthesitis in patients with BS without arthritis (BS-WA) is limited. Herein, we report a case of vascular BS complicated by enthesitis. In this case, heel pain was the dominant symptom at presentation. Laboratory tests revealed chlamydia antibody positivity, leading to a tentative diagnosis of reactive arthritis. Despite treatment, C-reactive protein (CRP) levels remained elevated. Imaging revealed numerous aneurysmal lesions in the large vessels. Based on these findings and other symptoms, patient was diagnosed with vascular BS. He tested positive for HLA-B15 and HLA-B46, which are associated with peripheral SpA. Subsequent remission induction therapy for BS was effective and the patient was discharged without complications. Our case and a literature review suggest that there exists a subgroup of BS-WA with a complication of enthesitis, possibly belonging to the spectrum of MHC-I-opathies. It is important to consider BS as a differential diagnosis in patients presenting with enthesitis and to conduct a precise medical history review regarding the symptoms of BS. [ABSTRACT FROM AUTHOR]

Historically inflammation against self was considered autoimmune which stems back to the seminal observations by Ehrlich who described serum factors, now known to be autoantibodies produced by B lineage cells that mediate “horror autotoxicus”. The 20th century elucidation of B- and T-cell adaptive immune responses cemented the understanding of the key role of adaptive immune responses in mediating pathology against self. However, Mechnikov shared the Nobel Prize for the discovery of phagocytosis, the most rudimentary aspect of innate immunity. Fast forward some 100 years and an immunogenetic understanding of innate immunity led to the categorising of innate immunopathology under the umbrella term ‘auto inflammation’ and terminology such as “horror autoinflammaticus” to highlight the schism from the classical adaptive immune understanding of autoimmunity. These concepts lead to calls for a two-tiered classification of inflammation against self, but just as innate and adaptive immunity are functionally integrated, so is immunopathology in many settings and the concept of an autoimmune to autoinflammation continuum emerged with overlaps between both. Herein we describe several historically designated disorders of adaptive immunity where innate immunity is key, including rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD) where the immunopathology phenotype is strongly linked to major histocompatibility complex (MHC) class II associations and responds to drugs that target T-cells. We also consider MHC-I-opathies including psoriasis and Behcet's disease(BD) that are increasingly viewed as archetype CD8 T-cell related disorders. We also briefly review the key role of barrier dysfunction in eczema and ulcerative colitis (UC) where innate tissue permeability barrier dysfunction and microbial dysbiosis contributes to prominent adaptive immune pathological mechanisms. We also highlight the emerging roles of intermediate populations of lymphocytes including gamma delta (gd) and mucosal-associated invariant T (MAIT) cells that represent a blend of adaptive immune plasticity and innate immune rapid responders that may also determine site specific patterns of inflammation. [ABSTRACT FROM AUTHOR]

The editorial "Microbiome and machine learning, volume II" published in Frontiers in Microbiology on October 15, 2024, explores the intersection of microbiomes and machine learning. It highlights the importance of understanding microbial communities for human health, biodiversity, and food production. The editorial discusses the challenges and benefits of using machine learning techniques, such as neural networks, in microbiome research. It also emphasizes the ethical considerations and practical applications of machine learning in various fields, including clinical diagnostics and food safety. [Extracted from the article]

The article discusses a multicentre study on the impact of cigarette smoking on Behcet's disease outcomes. The study included 445 Behcet's disease patients and found that smoking was associated with higher disease severity and visual impairment. Despite no significant difference in disease activity or remission rates between smokers and non-smokers, smokers had more severe damage. The study highlights the importance of considering smoking as a factor in managing Behcet's disease. [Extracted from the article]

Background: Behcet'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. Results: The 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. Conclusion: NFKB1 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. [ABSTRACT FROM AUTHOR]

Objective: The aim of this study was to investigate the phenotypes, predictive factors, and treatment approach of Behçet's patients with vascular involvement. Material and Method: This retrospective study analyzed 123 patients with Behçet's disease, 28 of whom had vascular involvement, and were followed up in our center. The study presented the vascular involvement patterns of the patients along with their clinical characteristics and comorbid conditions. The drugs usage by the patients were analyzed based on the first line and current medications, duration of medical therapy, and drug retention rate. Results: In Behçet's patients with vascular involvement, the male sex ratio was statistically higher compared to those without vascular involvement (60.7% vs 37.9%; OR=2.82 (1.17-6.77); p=0.018). The frequencies of Behçet's clinical manifestations, smoking, and comorbidities were similar in both groups. The most common subtype of vascular Behçet's is deep vein thrombosis (18; 64.2%), followed by superficial thrombophlebitis (5; 17.8%), neurovascular involvement (5; 17.8%), cardio-aortic (2; 7.1%) and pulmonary arterial (2; 7.1%). Azathioprine, glucocorticoids, and cyclophosphamide are the most preferred immunosuppressives in vasculo-Behçet's. Anticoagulant therapy was initiated in 67.8% (19; 28) of the patients at the first vascular event. Conclusion: The study presented that male gender predicts vascular involvement in Behçet's disease, with deep vein thrombosis being the most common vascular subtype. Although immunosuppressive drugs represent the cornerstone of treatment for vasculo-Behçet's disease, most patients had also received anticoagulant therapy following the initial attack. [ABSTRACT FROM AUTHOR]

Objective: 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. Methods: 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). Conclusions: 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. [ABSTRACT FROM AUTHOR]

The article discusses the history, clinical characteristics, diagnosis, genetic factors, pathophysiology, treatment, and prognosis of Adamantiades‐Behcet's disease (ABD), a chronic inflammatory disorder. ABD primarily affects the Eastern Mediterranean area and Central and East Asia, with a suspected autoimmune etiology. Diagnosis is based on clinical presentation, and treatment varies depending on the severity and organs involved. Biologic agents are increasingly used in managing severe and refractory cases of ABD. The article emphasizes the importance of multidisciplinary care and reference centers for optimal management of ABD. [Extracted from the article]

Objective: This study aims to assess the microstructural abnormalities in white matter (WM) among Behcet's disease (BD) patients, both with and without neurological involvement, utilising tract-based spatial statistics (TBSS) to elucidate the underlying causes of WM microstructural changes. Methods: This prospective study comprised 43 BD patients without neurological involvement, 15 neuro-Behcet's disease (NBD) patients with normal conventional MRI, and 54 healthy controls matched for age and sex. TBSS was applied in this diffusion tensor imaging study to conduct a whole-brain voxel-wise analysis of fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD), and radial diffusivity (RD) of WM. Results: Compared to the control group, BD patients exhibited decreased FA and increased MD and RD in nearly all WM tracts, along with increased AD in the left corticospinal tract (CST), left inferior longitudinal fasciculus (ILF), and left superior longitudinal fasciculus (SLF). NBD patients also showed a widespread decrease in FA and increased MD and RD, similar to BD patients without neurological involvement. Additionally, NBD patients had increased AD in the left CST, left ILF, left SLF, left inferior fronto-occipital fasciculus (IFOF), and right CST. Compared to BD patients without neurological involvement, NBD patients exhibited a greater reduction in FA and an increase in MD and RD in WM tracts, with no significant differences in AD. Conclusion: These results suggest that the main mechanism of microstructural changes in the WM of BD patients may be related to impaired fibre integrity, demyelination, and decreased myelin sheath integrity. Advances in knowledge: This study demonstrated BD patients without neurological involvement and NBD patients a decrease in FA and an increase in MD and RD were observed in larger areas of major WM tracts, while an increase in AD values was observed in fewer tracts. Our findings may be useful in understanding the pathophysiology underlying subclinical parenchymal involvement and neurological dysfunction in BD patients and the management of BD patients. [ABSTRACT FROM AUTHOR]

Behcet disease (BD) is a systemic vasculitis which can involve many different organ systems. Neurological involvement (NBD) occurs in 5.3% to 59% of BD patients. The diagnosis is challenging especially in case of inaugural neurological presentation, and is based on a constellation of clinical, laboratory, and neuroimaging findings. NBD can be subdivided into parenchymal NBD through an immune mediated meningoencephalitis with a predilection to the brainstem, basal ganglia, thalamus, cranial nerves, and spinal cord involvement, and extraparenchymal NBD encompassing cerebral veinous thrombosis and intracranial arterial involvement. Brain magnetic resonance shows ill-defined areas of oedema with high signal intensity on T2-FLAIR images, isointense or hypointense in T1-weighted images in the basal ganglia area or in the brainstem, which may extend to the diencephalic structures. Swelling might be noticed. Hemorrhages can be seen, such as contrast enhancement (blood brain barrier disruption). Magnetic resonance venography and computerized tomographic angiography can be used to diagnose extraparenchymal NBD. Treatment of parenchymatous forms is based on glucocorticoids associated with oral immunosuppressants (azathioprine, mycophenolate mofetil or methotrexate) in mild forms, and intravenous cyclophosphamide or infliximab in severe forms. The management of cerebral thrombosis consists of steroids course associated with an oral anticoagulation. An early recognition of this condition is mandatory to initiate adequate therapies in order to improve outcomes and limit the risk of sequelae, relapses, or death. The aim of this review is to summarize a comprehensive review on the various neurological presentations of BD with emphasizes on diagnostic tools, prognosis, and therapeutic issues. [ABSTRACT FROM AUTHOR]

Objectives To investigate the potential role of shelterin dysfunction in naïve CD4+ T cells in the pathogenesis of Behçet's disease (BD). Methods Naïve CD4+ T cells were isolated from 40 BD patients and 40 sex- and age-matched healthy controls (HC). Senescent profiles, shelterin subunits expression, telomere length, telomerase activity and critical DNA damage response (DDR) were evaluated. Telomere repeat factor-2 (TRF2) silencing was conducted for further validation. Results Compared with HC, BD patients had significantly decreased naïve CD4+ T cells, increased cell apoptosis, senescence, and productions of TNF-α and IFN-γ upon activation. Notably, BD naïve CD4+ T cells had shortened telomere, impaired telomerase activity, and expressed lower levels of shelterin subunits TRF2, TRF1- and TRF2-Interacting Nuclear Protein 2 (TIN2) and Repressor/Activator Protein 1 (RAP1). Furthermore, BD naïve CD4+ T cells exhibited significantly increased DDR, evidenced by elevated phosphorylated ataxia telangiectasia (AT) mutated (pATM), phosphorylated p53 (pp53) and p21. Finally, TRF2 silencing markedly upregulated DDR, apoptosis and proinflammatory cytokines production in HC naïve CD4+ T cells. Conclusion Our study demonstrated that TRF2 deficiency in BD naïve CD4+ T cells promoted cell apoptosis and senescence, leading to proinflammatory cytokines overproduction. Therefore, restoring TRF2 might be a promising therapeutic strategy for BD. [ABSTRACT FROM AUTHOR]

Objectives: 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. Methods: 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 3 months were invited to the clinic. Results: 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. Conclusion: 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. [ABSTRACT FROM AUTHOR]

BackgroundMethodResultsConclusionBehcet’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.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.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.Our data uncovered novel serum proteins that distinguish BD from RAS, which may potentially be useful in BD diagnosis and treatment. [ABSTRACT FROM AUTHOR]

Introduction: 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. Objective: 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. Methods: 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. Results: 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 2 years. Relapse rates were 4.5% at 1 year and 18.7% at 2 years, with disease progression observed in two cases. Adverse effects were primarily mild to moderate, with no reports of serious adverse events. Conclusion: 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. Key Points • Among the cohort of 53 patients diagnosed with Neuro-Behcet syndrome (NBS), parenchymal involvement was identified in 60.4% (n = 32), while non-parenchymal involvement was observed in 39.6% (n = 21) of cases. • Out of the 53 patients diagnosed with NBS, 20 individuals received a diagnosis of Behcet's syndrome upon their initial admission to our hospital due to central nervous system (CNS) involvement. • There were no discernible differences in the other clinical manifestations of Behcet syndrome between NBS patients requiring treatment with infliximab and those who did not necessitate such therapy. • Among patients with NBS who underwent treatment with ınfliximab, significant achievement of disease remission and stability was noted by the end of the second year, with noticeable prevention of disability accumulation and disease progression. [ABSTRACT FROM AUTHOR]

Background: Behcet's disease (BD) has a heterogeneous and unpredictable phenotype that differs in various geographical areas. Objective: To describe the clinical phenotype & outcome of Behcet's disease(BD) from Karnataka, India and compare them with large cohorts from endemic regions. Methods: Databases of practising rheumatologists from Karnataka were reviewed to retrieve clinical characteristics, course of illness, prescribing information and outcome at last follow-up of patients clinically diagnosed as BD. The classification criteria, namely revised International criteria for Behcet's disease (rICBD) and International study group (ISG) criteria were applied. Outcome was defined as complete or partial remission, persistent disease or relapse. Results: We included 72 patients, equal gender distribution and mean age 37.4 ± 12.8 years from 8 rheumatology centres. Commonest presentations were recurrent oral aphthosis 58(80.6%), genital ulcers 36(50%) and ocular manifestations 40(55.6%). Three-quarters [51/72(70.8%)] fulfilled rICBD criteria whereas only half [36/72(50%)] fulfilled ISG criteria. Apart from glucocorticoids [53/72(73.6%)], frequently prescribed therapies were colchicine 39(54.2%) and azathioprine 35(48.6%). Eleven-patients received biologics(anti-TNF-α) and JAK inhibitors to treat severe organ involvement. HLA-B*51 and pathergy tests were positive in 27/45(60%) and 12/34(35.3%) patients respectively. Outcomes were documented in 94.4%(68/72) patients at median follow-up of 24 (12;36) months. Majority [46/68(67.6%)] had complete remission, 17/68(25%) had partial remission, 4/68(5.9%) had persistent while 1/68(1.5%) had relapsing course. Conclusion: Majority of BD patients had orogenital aphthosis and ocular manifestations and an excellent response to treatment. Key Points • In our region, Behçet's Disease primarily manifests with recurrent oral aphthae and ocular involvement, with comparatively lower incidence of severe genital ulcers and neurological involvement than in endemic regions. • Apart from glucocorticoids, colchicine and azathioprine are the most commonly used agents. Biologics and JAK inhibitors are prescribed infrequently, primarily in cases of severe organ involvement. • A significant proportion of patients achieved either complete or partial remission during follow-up, with no observed mortality suggesting a milder disease course and better outcome compared to endemic regions. • Gender, HLA-B*51 status, and pathergy response did not exert any significant influence on the clinical profile or outcome in BD patients in Karnataka. [ABSTRACT FROM AUTHOR]

Purpose: Narrative Medicine complements the clinically centered approach, which focuses on the analytical and sanitary aspects, with the illness- and disorder- centered models, which deal respectively, with personal coping and social perception of a condition. Hypermobile Ehlers- Danlos Syndrome and Behçet's Disease are two rare multisystemic chronic disease experienced a myriad of clinical symptoms, psychological distress, and poor quality of life. The purpose of this report is to describe application of a multidisclipinary rehabilitation intervention according to the narrative medicine. Case description and Intervention: a 35-year-old woman with a 23-year history of Hypermobile Ehlers-Danlos Syndrome and Behçet's Disease was admitted. A multimodal rehabilitation approach was implemented and described through the patient's feelings and expected short-, medium-, and long- terms goals. Moreover, she reported her feeling with periodical interview by her therapist. History patient: The patient was seen for 32 weeks. A decreased pain, as well as improved function was recorded immediately post-intervention. Conclusion: Narration is not just an interpretation of the illness experience, but the result of reshaping the interpretations of the story that both patient and health professional. This discipline provides enrichment of care, especially when combined with rehabilitation in chronic disease, by paying attention to and using even in the therapeutic phase the stories of patients, family and health care personnel, giving the opportunity to define a tailored effectiveness rehabilitation approach at the light of the different points of view of the subjects. IMPLICATIONS FOR REHABILITATION: Narrative Medicine might play a key role in an interpretation of the illness experience to implement a multidisciplinary tailored rehabilitation approach. Narrative Medicine can help understand discomfort and difficulty associated with rehabilitation activities, thereby enhancing patient motivation and participation in interdisciplinary care. The Ehlers–Danlos Syndrome is a heterogeneous group of heritable connective tissue disorders representing a challenge in rehabilitation. Behçet's Disease is a rare multisystemic chronic disease experiencing an impaired function and a poor quality of life. A patient-oriented multimodal rehabilitation showed to be a useful approach in the case with both diseases, with a reduction of pain and an improvement of functioning. [ABSTRACT FROM AUTHOR]

Aim: The aim of this research is to evaluate the demographic attributes and clinical manifestations of uveitis in adult patients frequenting the Mansoura Ophthalmic Center. Methods: Utilizing a cross-sectional, prospective, analytical study design, this research engaged adult patients visiting the outpatient uveitis clinic at the Mansoura Ophthalmic Center. Comprehensive case evaluations involved collecting detailed patient histories, examining ophthalmic records, and conducting thorough ocular examinations. These examinations encompassed the assessment of visual acuity, slit-lamp examination, and fundus examination. Furthermore, selected cases underwent optical coherence tomography (OCT) and fundus fluorescein angiography (FFA). Results: The Study involved an examination of 411 eyes belonging to 254 uveitic patients. In the Egyptian context, anterior uveitis surfaced as the most prevalent form of uveitis. The average Best-Corrected Visual Acuity (BCVA) among the cases studied was 0.797 ± 0.77 LogMAR, with the majority of cases demonstrating vision superior to 0.3 LogMAR. Notably, the principal causes of vision loss were generally reversible. Macular edema was identified as the leading cause of vision loss, representing 20.7% of cases as evidenced by OCT. The ratio of non-infectious to infectious uveitis stood at 92.2% to 7.8%. The most commonly observed etiologies of non-infectious uveitis included Behçet's disease (33.3%), Vogt-Koyanagi-Harada (VKH) syndrome (19.7%), idiopathic causes (19.2%), and ankylosing spondylitis (AS) (11.9%). Conversely, the most frequent infectious etiologies were trematode-induced uveitis (2.9%), herpetic uveitis (1.7%), toxoplasmosis (1.5%), tuberculosis (TB) (1.5%), and brucellosis (0.2%). Conclusions: This study conclusively indicates that anterior uveitis is the predominant anatomical type of uveitis in Egypt. Further, etiological diagnoses of uveitis should particularly emphasize Behçet's disease, VKH syndrome, and ankylosing spondylitis. [ABSTRACT FROM AUTHOR]

Purpose: Determining uveitis etiology is a challenge. It is based primarily on demographic data and the characteristics of eye examination. It is not clear to what extent extraocular physical signs contribute to elucidating the etiology. This study aimed to establish the contribution of the clinical extra-ophthalmological features for the assessment of the underlying etiology of uveitis. Methods: We retrospectively reviewed 1307 patients with uveitis referred to our tertiary center between 2003 and 2021. Uveitis was classified according to the Standardization of Uveitis Nomenclature. Clinical features were collected at diagnosis by internists before the etiological diagnosis was made. The main outcome description was the contribution of clinical features. Results: Clinical extra-ophthalmological features contributed to the assessment of the underlying etiology of uveitis in 363 (27.8%) patients. The joint and the skin examinations were the most useful for etiological investigations, respectively in 12.3% and 11.8% of patients. Five etiologies of uveitis accounted for 80% of the cases: sarcoidosis, HLA-B27-related uveitis, Behçet's disease, multiple sclerosis, and Vogt-Koyanagi-Harada disease. Clinical extra-ophthalmological features were particularly important in the etiological diagnosis of acute bilateral anterior uveitis and panuveitis. Conclusion: This study suggests that clinical extra-ophthalmological features are essential for the etiological diagnosis of uveitis in more than a quarter of patients. It demonstrates once again the value of collaboration between ophthalmologists and other specialists experienced in performing extra-ophthalmological clinical examinations, particularly in patients with acute bilateral anterior uveitis and panuveitis. [ABSTRACT FROM AUTHOR]

Purpose: Behçet's syndrome (BS) is a chronic inflammatory disease affecting the small and large vessels of the venous and arterial systems and is characterized by recurrent oral and genital ulcers. Uveitis represents the most typical ocular manifestation and completes the triple symptom complex originally described. Recognized treatments for Behçet's uveitis (BU) include systemic glucocorticoids and immunosuppressive agents. No study has reported on the use of upadacitinib for BS with panuveitis. Herein, we report the use of upadacitinib in two patients with BU suffering from macular edema and persistent inflammation, which was refractory to systemic glucocorticoids and immunosuppressive agents. Methods: We retrospectively followed-up two cases, including an adolescent girl and a man in his thirties, with a 2- and 10-year history of BS, respectively. Results: Upadacitinib successfully treated BU, leading to improved visual acuity, controlled intraocular inflammation, and the disappearance of macular edema in both patients. The patients in this study were either recalcitrant to or intolerant to conventional therapy and adalimumab. Only the female patient revealed a mildly abnormal blood picture and slight transaminitis after 6 months of upadacitinib administration. However, no serious adverse events were reported in either of the two patients during follow-up. Conclusion: Upadacitinib can be considered an important future option for managing recurrent and recalcitrant cases of BU, especially in those with chronic ocular inflammation and macular edema, which are refractory to conventional therapies. [ABSTRACT FROM AUTHOR]

Evidence 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. Based 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. Forty-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. Frailty and pre-frailty are commonly detected even in younger patients with BS. Inflammation can be described as potential determinants of frailty status. [ABSTRACT FROM AUTHOR]

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 regression was used to 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 identified as 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. [ABSTRACT FROM AUTHOR]

Sexual health is an important part of a healthy life. The aim of this study is to define Behçet's sexual dysfunction and the factors affecting it. Sixty-nine patients with Behçet's disease (BD) and 74 healthy controls were included in the study. International Index of Erectile Function (IIEF), the Female Sexual Function Index (FSFI), the Beck Depression Inventory (BDI), and the Short Form Health Survey (SF-36) were filled out by patients and healthy control group (HCG). Follicle-stimulating hormone (FSH), luteinizing hormone (LH), prolactin and estradiol or testosterone levels according to gender were measured in Behçet's patients. The rate of sexual dysfunction and its relationship with gonadal hormones, Beck depression and SF 36 subgroups were evaluated in Behçet's patients. Sixty-nine patients with BD (mean age 39.55 ± 11.7) and 74 HCG (mean age 36.9 ± 6.84) were included in the study. Sexual dysfunction was observed in 74% (49) of BD and 59.5% (44) of HCG (p = 0.047). Prolactin level is within normal limits in all patients. Although there are abnormal levels of gonadal hormones, no relationship was found with sexual dysfunction. Depression especially is more prevalent compared to the healthy population (p = 0.016). The presence of depression negatively affects sexual life. Depression, SF 36 physical role limitations, energy vitality, vitality and mental health were associated with sexual dysfunction in patients with Behçet's disease. Sexual dysfunction was associated with the presence of depression and SF-36 subscales in Behçet's patients. [ABSTRACT FROM AUTHOR]

Behçet's disease (BD) is a multiorgan inflammatory vascular disease of unknown etiology, affecting vessels of any size and type. While, gastrointestinal involvement in BD is uncommon, esophageal Behçet's disease (EBD) is rarely reported, especially in the Mediterranean region. To report a case with esophageal stenosis as an initial presentation of BD and compare to reported cases worldwide. A 30-year-old Syrian male presented with dysphagia, odynophagia with weight loss 20 kg. He had a history of recurrent oral ulcers and was otherwise healthy. On examination he appeared pale, dehydrated with normal systemic examination. Blood tests revealed anemia (hemoglobin 10.5 mg/dl), elevated erythrocyte sedimentation rate (ESR) (90 mm/1sthr) and C-reactive protein (CRP)(98 mg/dl)levels. Upper gastrointestinal endoscopy showed mild ulceration with edema and mucosal thickness at the upper sphincter of the esophagus, leading to stenosis. Biopsy showed nonspecific inflammation, without granulomas or signs of malignancy. Percutaneous endoscopic gastrostomy (PEG) tube was placed and during hospitalization he developed genital ulcers and hemoptysis, with chest computed tomography revealing pulmonary vasculitis. The patient was diagnosed with BD, and treated with methylprednisolone and cyclophosphamide, leading to significant improvement. After two months he could swallow normally, and the PEG tube was removed with no recurrence after one year follow-up. He was maintained on azathioprine 100 g/day and prednisolone 7.5 mg/day. BD is a diagnostic challenge due to its diverse manifestations and geographic variations. EBD is rare and can cause disability. Early diagnosis of EBD and appropriate treatment are crucial for improving outcomes. [ABSTRACT FROM AUTHOR]

Objective: The goal of this study was to investigate the risk of preterm birth subtypes and hypertensive disorders of pregnancy in patients with systemic vasculitis using large, statewide databases. Methods: Births to nulliparous patients with prevalent systemic vasculitides (Takayasu arteritis [TAK], Behçet disease [BD], antineutrophil cytoplasmic antibody‐associated vasculitis [AAV], and Kawasaki disease [KD]) were identified using International Classification of Diseases, Ninth Revision codes in linked administrative data and birth records from the California Department of Health Care Access and Information and California Vital Statistics from 1991 to 2012. Hypertensive disorders of pregnancy and preterm delivery (PTD) subtypes were identified. Multivariable‐adjusted Poisson models estimated risk ratios (RRs) of these outcomes compared with the general birthing population without history of rheumatic disease. Results: A total of 96 births to nulliparous patients with systemic vasculitis were identified (TAK, 14; AAV, 31; BD, 26; KD, 15) and compared with 4,191,900 births of the nulliparous general population. Adjusted RRs for all PTD types were elevated in patients with vasculitis (RR 3.21, 95% confidence interval [CI] 2.15–4.79), as were the RRs of all PTD subtypes including preterm premature rupture of membranes (RR 4.30, 95% CI 2.05–9.01) and spontaneous PTD (RR 4.99, 95% CI 3.01–8.28). Of the spontaneous PTDs among patients with vasculitis, 16.7% were early PTDs (20–31 weeks), with the remaining 83.3% occurring between 32 to 36 weeks. Patients with vasculitis also had an elevated risk of hypertensive disorders of pregnancy (RR 2.96, 95% CI 1.72–5.10). Conclusion: Among first‐time births, we found that patients with systemic vasculitis have an elevated risk of PTD subtypes as well as hypertensive disorders of pregnancy. [ABSTRACT FROM AUTHOR]

Uveitis is a vision-threatening disease primarily driven by a dysregulated immune response, with retinal microglia playing a pivotal role in its progression. Although the transcription factor EGR2 is known to be closely associated with uveitis, including Vogt-Koyanagi-Harada disease and Behcet's disease, and is essential for maintaining the dynamic homeostasis of autoimmunity, its exact role in uveitis remains unclear. In this study, diminished EGR2 expression was observed in both retinal microglia from experimental autoimmune uveitis (EAU) mice and inflammation-induced human microglia cell line (HMC3). We constructed a mice model with conditional knockout of EGR2 in microglia and found that EGR2 deficiency resulted in increased intraocular inflammation. Meanwhile, EGR2 overexpression downregulated the expression of inflammatory cytokines as well as cell migration and proliferation in HMC3 cells. Next, RNA sequencing and ChIP-PCR results indicated that EGR2 directly bound to its downstream target growth differentiation factor 15 (GDF15) and further regulated GDF15 transcription. Furthermore, intravitreal injection of GDF15 recombinant protein was shown to ameliorate EAU progression in vivo. Meanwhile, knockdown of GDF15 reversed the phenotype of EGR2 overexpression-induced microglial inflammation in vitro. In summary, this study highlighted the protective role of the transcription factor EGR2 in AU by modulating the microglial phenotype. GFD15 was identified as a downstream target of EGR2, providing a unique target for uveitis treatment. [ABSTRACT FROM AUTHOR]

Background: Noncoding RNAs play pivotal roles in the process of autoimmune diseases. However, the definite contributions of these molecules to Behçet's disease (BD) are still unknown. This study aimed to explore the clinical value of a novel competing endogenous (ce) RNA network in the pathogenesis of BD and to assess its use in primary diagnosis. Methods: Bioinformatic analysis was applied to construct a BD-related ceRNA network: lncRNA (MIAT and PVT1)-miRNA (miR-93-5p and miR-124-3p)-mRNA (SOD-2 and MICA). Blood was obtained from 70 BD patients and 30 healthy subjects, and the serum expression of the tested RNAs was estimated via quantitative real-time PCR (qPCR). Serum tumor necrosis factor-alpha (TNF-α) levels were also determined. The associations between these RNAs were further analyzed, and receiver operating characteristic (ROC) curve and logistic regression analyses were employed to validate their diagnostic and prognostic values. Results: The expression levels of the lncRNAs PVT1 and miR-93-5p were significantly increased, whereas those of the lncRNAs MIAT and miR-124-3p, as well as those of the SOD-2 and MICA mRNAs, were significantly decreased in BD patients compared with controls. BD patients had significantly higher serum TNF-α levels than controls did. ROC curve analysis indicated that the selected RNAs could be candidate diagnostic biomarkers for BD. Moreover, the highest diagnostic efficiency was achieved with the combination of MIAT and miR-93-5p or PVT1 and miR-124-3p with either SOD-2 or MICA. Logistic regression analysis revealed that all RNA expression levels could be predictors for BD. Conclusion: Mechanistically, our research revealed a novel ceRNA network that is significantly disrupted in BD. The findings reported herein, highlight the noncoding RNA-molecular pathways underlying BD and identify potential targets for therapeutic intervention. These insights will likely be applicable for developing new strategies for the early diagnosis, management and risk assessment of BD as well as the design of novel preventive measures. Trial registration The protocol for the clinical studies was approved by Cairo University's Faculty of Pharmacy's Research Ethics Committee (approval number: BC 3590) [ABSTRACT FROM AUTHOR]

Background: Behcet's disease (BD) is an inflammatory disorder of unknown cause that is characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesions. Local vasculitis can cause damage to the visceral system, but it is rare in kidney patients, especially those with IgA nephropathy (IgAN). In China, a small number of related cases have been reported. Here we present a case of co-occurrence of BD and IgAN. Case presentation: : An 18-year-old female who presented with a history of recurrent oral ulcers was found ten years ago. Four years later, the patient presented with reddish nodules on the skin of both lower limbs and then presented with vulvar ulcers. This patient was clinically diagnosed with Behcet's disease after left calf skin biopsy and presented severe proteinuria and hematuria during this period. IgAN was diagnosed after percutaneous renal biopsy. The patient was treated with hormonal, anti-inflammatory, immunomodulatory, kidney protective, and protein-lowering urine agents. After 3 years of follow-up, the patient reappears oral ulcers, reddish nodules on the skin of both lower limbs and renal dysfunction. Conclusions: BD is less common in China and is clinically prone to missed diagnosis and misdiagnosis. BD with IgAN is rarer. We should regularly pay attention to the routine urine and renal function of BD patients for early detection and treatment and to prevent further progression of the disease. [ABSTRACT FROM AUTHOR]

Objective Granulomatosis with polyangiitis (GPA) is an ANCA-associated vasculitis. The 2022 ACR/EULAR-endorsed classification criteria for GPA was derived using data only from adult patients. We aimed to assess the performance of the ACR/EULAR classification criteria for GPA in paediatric patients and compare it with the EULAR/Pediatric Rheumatology International Trials Organization (PRINTO)/Pediatric Rheumatology European Society (PReS)-endorsed Ankara 2008 criteria for GPA. Methods Retrospective data of paediatric patients with GPA in 20 centres from 9 countries were evaluated. The diagnosis of GPA was made according to the expert opinion. The sensitivity, specificity, positive predictive value, and negative predictive value of the criteria sets were evaluated. Results The study included 77 patients with GPA and 108 controls [IgA vasculitis (n  = 44), Takayasu's arteritis (n  = 20), microscopic polyangiitis (n  = 16), polyarteritis nodosa (n  = 14), Behçet's disease (n  = 12), eosinophilic granulomatosis with polyangiitis (n  = 1) and Cogan's syndrome (n  = 1)] with a median age of 17.8 and 15.2 years, respectively. Among patients with GPA, constitutional symptoms (85.7%) and ENT involvement (79.2%) were the most common presentations. In the GPA group, 73 patients fulfilled the Ankara 2008 criteria and 69 the ACR/EULAR classification criteria. Sensitivities of the Ankara 2008 criteria and the ACR/EULAR classification criteria were 94.8% and 89.6%, while specificities were 95.3% and 96.3%, respectively. No significant difference was found between sensitivities and specificities of both classification criteria (P  = 0.229 and P  = 0.733, respectively). Conclusion In children, both the ACR/EULAR and EULAR/PRINTO/PReS Ankara 2008 classification criteria for GPA perform well and similarly. [ABSTRACT FROM AUTHOR]

Objective Patients with Behçet's disease (BD) may experience long-term morbidity caused by various forms of cardiovascular disease. This study aimed to assess the risk for cardiovascular comorbidity in paediatric BD patients with and without vascular involvement, independent of the contribution of traditional risk factors. Methods Paediatric patients classified as having BD according to the 2015 Peadiatric BD (PEDBD) criteria were included in the study. Twenty-four-hour ambulatory blood pressure monitoring (ABPM), transthoracic echocardiography, and carotid intima-media thickness (cIMT) measurements were performed. Patients with an active disease or those who have other known risk factors for cardiovascular disease were not included in the study. Results Thirty-one children and adolescents with paediatric BD (16 female, 51.6%; F/M: 1.06) were enrolled in the study. Among the BD patients, 10 patients (34.4%) had abnormal ABPM. Carotid IMT values, mean arterial pressure, systolic and diastolic blood pressure by ABPM and the prevalence of abnormal ABPM, non-dipping, and ambulatory hypertension were similar between patients with and without vascular involvement. The echocardiography measurements showed that BD patients with vascular involvement had a significantly higher velocity and velocity time integral of the left ventricle outflow tract, which may indicate increased stiffness of the aorta. Conclusion Paediatric BD patients with vascular involvement may tend to have more cardiovascular risk factors. However, cardiovascular assessment should be considered in all BD patients, regardless of the involved systems. We suggest that ABPM may accurately define hypertension and cardiovascular risk in BD. [ABSTRACT FROM AUTHOR]

Aim: Behcet's disease (BD) is a systemic disorder characterized by vasculitis, resulting in thickened vascular walls that reduce elasticity and impair function. BD can involve the cardiovascular system in three ways: cardiac, arterial, and venous. In this study, our objective was to evaluate the efficacy of pulmonary arterial stiffness (PAS) and pulmonary pulse transit time (PPTT) measures in demonstrating right ventricular functions in asymptomatic BD patients. We aimed to objectively evaluate right ventricular function in patients with BD using four‐dimensional echocardiography (4DE). Method: This study included 40 patients diagnosed with BD and 40 healthy subjects. Demographic, clinical, laboratory, and echocardiographic parameters were compared. In addition to standard transthoracic echocardiographic evaluation, right ventricle quantification (RVQ) by using the 4DE and 2D‐speckle tracking echocardiography were performed. Results: The sPAP, 4D RVQ, and right ventricular strain values exhibited significant differences between the BD and control groups. Right ventricular end‐diastolic diameter (RVDD), right ventricular end‐systolic diameter (RVSD), right atrium (RA) area, right ventricular myocardial performance index (RVMPI), and PAS were increased in BD patients compared to the control group. Right ventricular ejection fraction (RVEF), right ventricular fractional area change (RVFAC), tricuspid annular plane systolic excursion (TAPSE), Tricuspid S', and PPTT were decreased in BD patients compared to control subjects. PPTT correlated with right ventricular free wall strain (RV‐FWS) and PAS. In a multivariate linear regression analysis, PAS and RVFAC were found to be independent predictors of RVFWS. In addition, RVFAC and TAPSE are independent predictors for PPTT. Conclusion: Patients with BD may have elevated pulmonary arterial stiffness (PAS) in correlation with decreased PPTT. To ascertain the prognosis for these individuals, right ventricular (RV) functions must be evaluated. Measurements of RVFAC and RVEF via 4DE and deformation imaging techniques may be more useful in identifying subclinical impairment of RV. Individuals with BD, PAS, and PPTT may suggest a link between early pulmonary vascular remodeling and RV subclinical impairment. [ABSTRACT FROM AUTHOR]

Objectives: Post-thrombotic syndrome (PTS) is a frequent and important consequence of deep vein thrombosis (DVT) for Behcet's disease (BD) patients. Although various clinical scales are used to diagnose PTS, Villalta scale was accepted as the standard tool to diagnose and grade the severity of PTS. Poor quality of life (Qol) in the general population was defined for patients with PTS, however, studies in BD patients with PTS is limited. Our aim was to compare the performance of different scales to assess venous disease in BD patients with a history of DVT and to assess the relationship with quality of life. Methods: Patients with BD (n = 194, M/F:157/37, age:39.1 ± 9.5 years) with a DVT history were investigated. Villalta, VCSS,CEAP scale and SF 36,Veines scales were used to assess venous disease and QoL respectively. Results: Among BD patients, 120 (61.9 %) patients were classified as having PTS by Villalta and of patients 18% had severe PTS. Half of patients with CEAP score <4 were classified as having PTS. Also, 42% of patients with CEAP>4 and almost two third of VCSS classified severe CVD patients was grouped in severe PTS by Villalta scale. VCSS and Villalta classified PTS patients had decreased disease specific and general Qol scores compared to the patients without PTS. Also, severe PTS group (by VCSS) had decreased veines QoL scores and PCS compared to mild/moderate group. Conclusion: BD patients with DVT have a high risk of PTS. Our results show that both Villalta scale and VCSS should be used to assess venous disease BD patients with DVT. However, VCSS classified severity of PTS can show better correlation with venous disease -specific QoL. [ABSTRACT FROM AUTHOR]

Background/Objectives: Neuro-Behçet's disease (NBD) is one of the most severe complications of Behçet's disease (BD). The incidence of NBD varies widely worldwide. This study aimed to estimate its current incidence in Northern Spain. Methods: This was a retrospective population-based cohort study of 120 patients in Northern Spain diagnosed with BD according to the 2013 International Criteria for BD (ICBD) between 1 January 1999 and 31 December 2019. NBD diagnoses were made according to International Consensus Recommendation (ICR) criteria. Overall, 96 patients were included, and their demographic and clinical data were collected. The incidence of NBD was estimated by age, gender, and year of diagnosis between 1999–2019. Results: NBD was diagnosed in 23 of 96 (24%) patients (15 women/8 men) (mean age: 44 ± 13.9 years). HLA-B51 was positive in 5 of 13 (38.4%) cases tested. A total of 10 (43.5%) patients had parenchymatous NBD, 10 (43.5%) had non-parenchymatous NBD, and 3 (13%) had mixed NBD. Incidence during the study period was 0.13 (95% CI, 0.11–0.26) per 100,000 people-years. There were no significant differences in gender in the incidence rate stratified by age (p > 0.05). Furthermore, there was a linear relationship with a mild decrease in age at diagnosis over time. Conclusions: Epidemiological characteristics of NBD in Northern Spain are similar to those of neighboring countries, except female gender predominance. [ABSTRACT FROM AUTHOR]

Aims: This study investigated histamine intake and its associations with clinical and biochemical findings in patients with Behçet syndrome. Methods: Patients with Behçet syndrome were prospectively enrolled using a crosssectional, multicenter, and online survey design. Sociodemographic parameters, including age, gender, smoking and alcohol intake, nutritional counseling history, anthropometric measurements, clinical characteristics, and biochemical results, were obtained using an online questionnaire. Dietary histamine intake was determined using a food frequency questionnaire. Results: The study included 66 patients (mean age: 37.5±11.3 years, women: 53%. Food consumption was reported to trigger oral aphthae in 81% of the individuals, and the most frequently reported triggers were eggplant (37.5%), tomatoes (37.5%), and citrus fruits (34.3%). There was a significant positive correlation between dietary histamine intake and white blood cell counts (r=0.650; p=0.050). There were no significant differences in the clinical characteristics, including oral aphthae, genital ulcers, uveitis, dermatologic lesions, gastrointestinal system involvement, joint involvement, and vascular involvement between patients with low and high dietary histamine intake. A positive correlation was found between dietary histamine intake and the frequency of attacks (r=0.324; p=0.008). Conclusions: This study showed that increased dietary histamine intake was associated with an increased frequency of attacks in patients with Behçet syndrome. Oral aphthae are associated with certain foods, such as eggplant, tomatoes, and citrus fruit. [ABSTRACT FROM AUTHOR]

Objectives: We aimed to present the demographic, clinical, laboratory, and treatment data of children with non-infectious uveitis and to evaluate the risk factors for the development of complications and the need for biological treatment. Method: Patients diagnosed with non-infectious uveitis in childhood and followed up for at least 1 year were included in the study. Demographic data, including age, gender, age at diagnosis, uveitis in first-degree relatives, and rheumatologic diseases, were obtained retrospectively from medical records. The presence of complications or the need for biologic therapy was considered a composite outcome suggesting severe disease. Results: The study included 123 patients (female: n = 59, 48%). The mean age at diagnosis was 14.89 ± 4.86 years. Uveitis was symptomatic in 104 patients (84.6%). Approximately one-quarter of the patients had at least one rheumatic disease (n = 35, 28.5%), the most common being juvenile idiopathic arthritis. Thirty-three patients (26.8%) had anti-nuclear antibody positivity. Biologic agents were needed in 60 patients (48.8%). Complications developed in 14 patients (11.4%). Early age at disease onset (aOR, 0.875; 95% C.I. 0.795–0.965, p = 0.007) and female gender (aOR, 2.99; 95% C.I. 1.439–6.248, p = 0.003) were significantly associated with the need for biologic treatment, while Behçet's disease (BD) was strongly associated with uveitis-related complications (aOR, 14.133; 95% C.I. 2.765–72.231, p = 0.001). Conclusion: We suggest that among pediatric patients with non-infectious uveitis, females, those with an early age of disease onset, and those with BD need to be closely monitored due to a significantly increased risk of severe disease. Key Points • Limited data exist on the clinical course of non-infectious uveitis in children and the associated risk factors for severe disease. • Our study reveals that nearly a quarter of pediatric patients with non-infectious uveitis also have a rheumatic disease. • Among pediatric patients diagnosed with non-infectious uveitis, we observed an increased risk of severe disease in those with an earlier onset age, in female patients, and in those diagnosed with Behçet's disease. [ABSTRACT FROM AUTHOR]

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Behçet's disease (BD) is a systemic disease with unknown etiopathogenesis and varying disease presentations. Fatty acids (FA) are essential biological compounds that are involved in complex metabolic pathways. They may contribute to inflammation and endothelial dysfunction by participating in many signaling pathways. Increased FAs levels are associated with an increased risk for various diseases. This study aimed to determine the relationship between FA, BD, and thrombotic complications. A total of 97 patients were recruited from the rheumatology department of a single center as a case–control study. The participants were divided into three groups: 36 patients with BD with thrombosis (Group 1), 24 patients with BD without thrombosis (Group 2), and 37 age‐ and sex‐matched controls (Group 3). The analysis of 37 different FA with carbon numbers in the range of (4:0) and (24:1) in the samples were analyzed and compared between groups. Myristic acid (MA), methyl eicosatrienoate, and stearic acid (STA) levels were found to be significantly higher in BD with thrombosis than in BD without thrombosis, and palmitic acid (PA) levels were significantly higher in BD with thrombosis than in healthy individuals. MA was found to be a significant marker for differentiating between thrombotic BD. PA and STA are important markers for detecting thrombotic BD. In BD, lipotoxicity created by FA, such as PA, STA, and MA, plays a role as an inducer of inflammation and thrombosis through various mechanisms. [ABSTRACT FROM AUTHOR]

The given text provides summaries of various research studies presented at the Middle East Immunology Summit. The first study discusses a case of Evan syndrome secondary to systemic lupus erythematosus (SLE) in a pediatric patient. The second study examines the impact of COVID-19 vaccination on circulating cytokine profiles in individuals in Saudi Arabia. The third study explores the prevalence of food and inhalation allergen-specific IgE antibody positivity in residents of the Northern Emirates of the UAE, highlighting differences between Arab and non-Arab individuals. These studies contribute to our understanding of autoimmune diseases, vaccine responses, and allergies in the Middle East region. [Extracted from the article]

Background Behçet's disease is an inflammatory condition, caused by vasculitis of big and small veins and arteries in which, although vascular inflammation is the basis of disease, cardiac involvement is rare. We present a rare case of a man, affected by Behçet's disease, with pulmonary embolism due to a floating thrombus in the right ventricle. Case summary We report a case of a 36-year-old man admitted to emergency department due to dyspnoea and haemoptysis. He had already been diagnosed with Behçet's disease, and he was in therapy with low doses of azathioprine and prednisone from three months. Thorax CT scan detected pulmonary embolism with pulmonary infraction. No evidence of deep vein thrombosis was found. The echocardiogram pointed out a floating mass of at least 30 mm in the right ventricle. Cardiac magnetic resonance confirmed the diagnosis of right ventricle thrombosis. On the hypothesis of an inflammatory genesis of the thrombosis, immunosuppressive drugs and anticoagulation with vitamin K antagonist were prescribed. The patient underwent echocardiograms every 3 weeks, and the mass disappeared 5 months later. Discussion Behçet's disease is a systemic inflammatory disorder that often affects vessels and rarely the heart. Thrombosis can be the only clinical feature of primary or relapsing events with also atypical origin site. Thrombosis suggests a high inflammatory status that needs to be balanced with the right immunosuppressive therapy, associated to anticoagulation. [ABSTRACT FROM AUTHOR]

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

Behcet's disease is a multisystem inflammatory disorder, whose vascular involvement is very rare. Aneurysmal arterial involvement is the severe form of the disease, it constitutes a therapeutic challenge given its severity, and frequent secondary complications. Profunda femoral artery aneurysms (PFAAs) are extremely rare. The systematic immunosuppressive drug treatment and endovascular or surgical technique thoroughness can reduce relapse rate. We report the case of a young man in whom a false aneurysm of the profunda femoral artery revealed Behcet's disease. He underwent an endovascular treatment by embolization using coils; with good control after 3 months.

Abstract Background Behcet’s disease (BD) is an inflammatory disorder of unknown cause that is characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesions. Local vasculitis can cause damage to the visceral system, but it is rare in kidney patients, especially those with IgA nephropathy (IgAN). In China, a small number of related cases have been reported. Here we present a case of co-occurrence of BD and IgAN. Case presentation : An 18-year-old female who presented with a history of recurrent oral ulcers was found ten years ago. Four years later, the patient presented with reddish nodules on the skin of both lower limbs and then presented with vulvar ulcers. This patient was clinically diagnosed with Behcet’s disease after left calf skin biopsy and presented severe proteinuria and hematuria during this period. IgAN was diagnosed after percutaneous renal biopsy. The patient was treated with hormonal, anti-inflammatory, immunomodulatory, kidney protective, and protein-lowering urine agents. After 3 years of follow-up, the patient reappears oral ulcers, reddish nodules on the skin of both lower limbs and renal dysfunction. Conclusions BD is less common in China and is clinically prone to missed diagnosis and misdiagnosis. BD with IgAN is rarer. We should regularly pay attention to the routine urine and renal function of BD patients for early detection and treatment and to prevent further progression of the disease.

Background/Aims The association between inflammatory bowel disease (IBD) and gallstone and renal stone formation has been established. However, few studies have investigated this association in patients with intestinal Behçet’s disease (BD). We aimed to examine the prevalence of gallstones and renal stones in patients with intestinal BD and identify potential risk factors. Methods We analyzed gallstone and renal stone occurrences in 553 patients diagnosed with intestinal BD who had undergone cross-sectional imaging examinations between March 2005 and April 2021 at the IBD Center, Severance Hospital, Seoul, South Korea. Logistic regression models were used to identify risk factors for gallstone and renal stone formation. Results Of 553 patients over a mean 12.1-year duration, 141 (25.4%) patients had gallstones and 35 (6.3%) had renal stones. In multivariate logistic regression analysis, disease duration > 19 years (OR 2.91, 95% CI 1.56–5.44, p < 0.001), prior intestinal BD-related surgery (OR 2.29, 95% CI 1.42–3.68, p < 0.001), and disease activity index for intestinal BD scores ≥ 75 (OR 2.23, 95% CI 1.12–4.45, p = 0.022) were associated with increased gallstone occurrence. A positive correlation was observed between renal stones, disease duration > 19 years (OR 5.61, 95% CI 1.98–15.90, p = 0.001) and frequent hospitalization (> 3 times) (OR 3.29, 95% CI 1.52–7.13, p = 0.002). No significant correlation was observed between gallstone and renal stone occurrence. Conclusions These findings contribute to greater understanding concerning gallstone and renal stone prevalence and associated risk factors in patients with intestinal BD.

Rong Liu,1,&ast; Tingfen Ji,1,2,&ast; Yixia Jiang,1 Hequan Li1 1Department of Respiratory Medicine, The First Affiliated Hospital of Zhejiang University School of Medicine, Hangzhou, Zhejiang, People’s Republic of China; 2Department of Respiratory and Critical Care Medicine, Lishui People’s Hospital, Lishui, Zhejiang, People’s Republic of China&ast;These authors contributed equally to this workCorrespondence: Hequan Li, Email lihequan@zju.edu.cnPurpose: Recurrent inflammatory fevers with multisystem involvement occur clinically and may indicate an autoimmune disease.Case: We present a young male diagnosed with pulmonary embolism who experienced recurrent fever during hospitalization and was unresponsive to antibiotics and antipyretics. A follow-up history revealed chronic oral and genital ulcers, leading to a final diagnosis of Behçet’s disease.Conclusion: The patient’s temperature normalized rapidly after corticosteroid therapy, and infection markers returned to normal. Complete remission was achieved with immunosuppression and glucocorticoid therapy. Reporting characteristics, treatment experience, and outcomes of such cases are essential to inform future diagnosis and management strategies.Keywords: fever, Behçet’s disease, pulmonary embolism, atrial mass