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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]

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]

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]

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]

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]

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 cross-sectional, 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.

This study employed bibliometric analysis to examine the current literature on Behcet Syndrome, an autoimmune disorder with complex pathophysiology and inadequate therapeutic options. The researchers collected 3,462 publications related to Behcet Syndrome from 2010 to 2021 via PubMed and conducted co-word and social network analyses to identify research hotspots and potential future directions. The co-word analysis produced a bibliographic data matrix, which revealed 72 high-frequency medical topic title (MeSH) terms. Using repeated dichotomy in the gCLUTO software, the researchers created a visualization matrix that classified the hot topics into six categories over the 12-year study period. The first quadrant contained six mature and well-developed research topics, including biological therapy, immunosuppressive agents, clinical manifestations, complications of Behcet Syndrome, Behcet Syndrome diagnosis, and aneurysm etiology and therapy. The third quadrant comprised four research topics with potential for growth, including Behcet Syndrome genetics and polymorphism, immunosuppressive agents, biological therapy and heart diseases, and thrombosis etiology. The fourth quadrant encompassed the pathophysiology of and quality of life in Behcet Syndrome as well as psychology. In the social network analysis, the researchers identified potential hotspots based on subject keywords close to the network's edge. These included genetic association studies, antibodies, genetic predisposition to disease/genetics, and monoclonal and humanized therapeutic use. Overall, this study's bibliometric analysis of Behcet Syndrome literature from the past 12 years identified unexplored subjects and developing hot spots that could lead to potential research directions for Behcet Syndrome. [ABSTRACT FROM AUTHOR]

Objectives: Behçet uveitis (BU) is a potentially blinding disorder. The main determinant of visual prognosis is early and appropriate treatment that provides rapid suppression of inflammatory attacks, control of subclinical inflammation, and prevention of new attacks. Our study aimed to determine the Turkish uveitis specialists’ approach regarding the treatment choices and management of special situations such as pregnancy, vaccination, and surgical planning in BU patients, and to increase information sharing and raise awareness of issues where knowledge is lacking. Materials and Methods: A web-based survey including 16 questions about the treatment approach in ocular involvement of Behçet’s disease was sent via e-mail to uveitis specialists in Türkiye. Based on the answers of 49 ophthalmologists who responded to the survey, we evaluated the approaches of uveitis specialists in our country to initiating treatment, selecting therapeutic agents, monitoring, switching and stopping treatment, and special situations such as surgical planning, vaccination, and pregnancy in BU patients. Results: Uveitis specialists in our country mostly act in accordance with the guidelines in the decision to start treatment, selection of therapeutic agents, and monitoring the safety of treatment in BU. However, there is a lack of information about the therapeutic approach in pregnancy and vaccination practices. It was also observed that there is no consensus on the precautions to be taken before cataract surgery. Conclusion: Our study has shown that there is a need for more detailed and widespread information sharing on treatment in preparation for ocular surgery, safety monitoring, drug use during pregnancy, and vaccination in BU patients.

Objectives: Behçet uveitis (BU) is a potentially blinding disorder. The main determinant of visual prognosis is early and appropriate treatment that provides rapid suppression of inflammatory attacks, control of subclinical inflammation, and prevention of new attacks. Our study aimed to determine the Turkish uveitis specialists' approach regarding the treatment choices and management of special situations such as pregnancy, vaccination, and surgical planning in BU patients, and to increase information sharing and raise awareness of issues where knowledge is lacking. Materials and Methods: A web-based survey including 16 questions about the treatment approach in ocular involvement of Behçet's disease was sent via e-mail to uveitis specialists in Türkiye. Based on the answers of 49 ophthalmologists who responded to the survey, we evaluated the approaches of uveitis specialists in our country to initiating treatment, selecting therapeutic agents, monitoring, switching and stopping treatment, and special situations such as surgical planning, vaccination, and pregnancy in BU patients. Results: Uveitis specialists in our country mostly act in accordance with the guidelines in the decision to start treatment, selection of therapeutic agents, and monitoring the safety of treatment in BU. However, there is a lack of information about the therapeutic approach in pregnancy and vaccination practices. It was also observed that there is no consensus on the precautions to be taken before cataract surgery. Conclusion: Our study has shown that there is a need for more detailed and widespread information sharing on treatment in preparation for ocular surgery, safety monitoring, drug use during pregnancy, and vaccination in BU patients. [ABSTRACT FROM AUTHOR]

PurposeMethodsResultsConclusionThe prognostic importance of vitreous cells (VC) in patients with Behçet syndrome (BS) is unknown. We aimed to determine the frequency of developing posterior uveitis (PU) and any additional risk factors associated with the development of PU in BS patients with VC at diagnosis.The charts of 572 consecutive BS patients who were registered between 2010 and 2012 were reviewed. Among the patients with a follow-up of ≥2 years, we included 110 patients with VC in one or both eyes and 147 patients without any eye findings in both eyes at baseline and compared them for the development of PU.Among the 110 included patients with VC, 61 had VC in both eyes, 34 had VC in only one eye, and 15 had VC in one eye and PU in the other eye. There was anterior uveitis (AU) in addition to VC in the same eye in 13 patients at baseline. PU developed in 24 (22%) of these patients during a mean follow-up of 1.9 ± 1.1 years. This was significantly more frequent than the 147 patients without any eye findings at baseline, among whom there were only 2 patients who developed PU (p < 0.001). Multivariate logistic regression analysis showed that having AU in addition to VC in the same eye (OR, 5.03, 95% CI; 1.37–18.47) was an independent risk factor for the development of PU in patients with VC.Careful follow-up is required for patients with VC at diagnosis, since 22% developed PU within 2 years. [ABSTRACT FROM AUTHOR]

Objective: Rare disease. Background: Pulmonary artery aneurysm (PAA), defined as a pathologic dilatation of the PA greater than 1.5-fold the normal diameter, is a rare complication of Behçet disease. It is due to a weakening of the vessel wall for a great vessels' vasculitis, often asymptomatic and incidentally diagnosed on imaging studies. However, if ignored, it can lead to life-threatening complications such as rupture and massive hemoptysis. We report the case of a giant fast-growing PAA in a young patient with a history of Behçet disease in which an inadequate follow-up and poor patient information could had led to life-threatening complications. Case Report: A 37-year-old man with a history of Behçet disease presented to our Emergency Department with hemoptysis due to a right inferior lobar artery aneurysm measuring 52×33 mm. The aneurysm was detected years before, measuring 18 mm, but the patient and physicians missed the subsequent follow-up. After several attempts at embolization, the multidisciplinary board suggested to proceed with surgical intervention. Surgery was performed with an extracorporeal circulation system kept on stand-by due to the high hemorrhagic risk. By opening the fissure, the dilatation of the inferior lobar artery was clearly identified up to the origin of the middle lobar branch. Thus, a lower-middle bilobectomy was performed after the introduction of a suction cannula in the aneurysm, which facilitated its emptying and the subsequent maneuvers. Conclusions: PAA is a rare disease, generally treated with medical therapy or interventional procedures. However, giant and fast-growing aneurysms are more likely to entail complications and often required immediate treatment. In this case, primary surgical intervention with a pulmonary bilobectomy appeared mandatory to avoid life-threatening events. [ABSTRACT FROM AUTHOR]

Background While biologic therapy, typically with infliximab or Roferon, was used for Behçet syndrome after first-line immunosuppressants, no high-quality randomised trials or predictive biomarkers were available. Objective To undertake a randomised controlled clinical trial of infliximab versus Roferon in Behçet syndrome and identify potential biomarkers for response. Design Pragmatic, standard of care, single-masked, randomised, two-arm, parallel head-to-head trial, with exploratory study on potential role of interferon lambda 3 and interferon lambda 4 single nucleotide polymorphisms and urinary metabolomics biomarkers. Setting Three national UK Behçet syndrome centres and allied clinics. Participants Patients with active Behçet syndrome, fulfilling International Study Group 1990 criteria, with inadequate response to or intolerance of first-line treatment. Intervention Randomisation to infliximab (5 mg/kg intravenous infusion) or Roferon (subcutaneous injection), utilising the UK Behçet syndrome drug pathway protocol. Outcomes Primary outcome: modified Behçet’s disease activity index at 12 weeks of therapy. Secondary outcomes: (1) modified Behçet’s disease activity index score at 24 weeks and (2) significant improvement at 12 and 24 weeks from baseline in vitreous haze and best corrected visual acuity change, oral ulcer severity score, number of genital ulcers, arthritis pain, adverse events, reduction in dose of glucocorticoid, quality-of-life scores and Physician’s Global Assessment of disease activity. Sample size Utilising a Bayesian analysis of covariance model (80% credible interval), initial sample size was 45/arm (Bayesian power 90%). With an anticipated 10% dropout rate, 100 patients were to be recruited. Following recommendations to reduce the overall length of the trial, this was revised down to 80 patients (36 in each arm, allowing for 10% dropout): 80% equi-tailed credibility interval, Bayesian power 88%. In total, 79 patients were eventually recruited for the study. Methods Patients with refractory active Behçet syndrome underwent stratified block randomisation, based on randomly permuted blocks with random block sizes of two and four, allocating treatment to either infliximab or Roferon. Follow up with symptom-directed examination at weeks 12 and 24 according to standard of care. Analysis of the primary end point was undertaken using a Bayesian analysis of covariance approach. Informative priors for the anticipated treatment effect were derived from a cohort of six international experts prior to the start of the study. Results In this first prospective head-to-head randomised controlled clinical trial of two biologic drugs in Behçet syndrome, both infliximab and Roferon were equally effective [mean difference (80% credibility interval) = 0.13 (–0.19 to 0.46)], with a trend for minor benefit in favour of infliximab in terms of tolerability and treatment persistence. Genetic data suggested a potential association between patient outcome and carriage of either rs4803221 or rs7248668 variants in the interferon lambda 3 (interleukin 28B) gene locus in the Roferon-treated arm. However, with the relatively small sample size, statistical significance of the association was lost when correcting for multiple tests. Metabolomic analysis identified potential markers of a metabolic response to treatment with infliximab. Limitations Single-masked design. Slow recruitment with fewer patients recruited in total, limiting the strength of analysis for secondary outcomes and mechanistic studies. Conclusion We report clinical efficacy in both infliximab and Roferon in refractory active Behçet syndrome, together with the potential for a novel metabolomic biomarker identifying response to infliximab. Future work Further work will characterise the appropriate metabolite(s) from existing samples to inform future prospective trials to study this in more detail clinically. The efficacy of Roferon in Behçet syndrome may support future manufacture of this drug. Trial registration This trial is registered as EudraCT Number: 2014-005390-36; ISRCTN49793874. Funding This award was funded by the National Institute for Health and Care Research (NIHR) Efficacy and Mechanism Evaluation (EME) programme (NIHR award ref: 12/205/46) and is published in full in Efficacy and Mechanism Evaluation; Vol. 11, No. 17. See the NIHR Funding and Awards website for further award information. Plain language summary Behçet syndrome, a very rare disease in the UK, causes major illness. Yet without high-quality, randomised, controlled trials, choosing treatment is somewhat hit and miss. We set up a randomised controlled clinical trial to study the two most widely used biologic drugs in Behçet syndrome, infliximab and Roferon, head-to-head and searched for potential blood and urinary markers for response. Patients with active Behçet syndrome, in the United Kingdom national Behçet syndrome centres and allied clinics, who had not responded to or could not tolerate first-line treatment were randomised to either infliximab infusions or Roferon injections. The primary outcome was modified Behçet’s disease activity index at 12 weeks of therapy. Secondary outcomes included modified Behçet’s disease activity index at 24 weeks and significant improvements in individual organs, quality of life and Physician’s Global Assessments of activity at 12 and 24 weeks. Initial assessment suggested 100 patients were required for a statistically meaningful outcome but was revised down to 80 following recommendations to shorten the trial. In this first prospective head-to-head randomised controlled clinical trial of two biologics in Behçet syndrome, both drugs worked equally well. There was a non-significant trend for minor benefits of infliximab in terms of tolerability and treatment persistence. Genetic data suggested a potential association between patient outcome and carriage of either rs4803221 or rs7248668 variants in the interferon lambda 3 (interleukin 28B) gene locus in the Roferon arm, but statistical significance was lost with the relatively small sample size. Metabolomics analysis identified potential markers of a metabolic response to infliximab. The limitations of the study included the single-masked design: patients (but not clinicians) were aware of their treatment, and fewer patients were studied than planned. This limited the strength of analysis for secondary outcomes and mechanistic studies. We now plan to characterise the metabolite(s) from existing samples to design future trials to study if there can be effective targeting of treatment in Behçet syndrome. Scientific summary Background Behçet syndrome (BS), a multisystem inflammatory vasculitis, is infrequent in the UK, but it has the potential to cause significant morbidity and mortality. The evidence base supporting biologic treatment, which is used for active disease after failure of standard immunosuppression or when prognosis is poor, is largely based on uncontrolled studies. At the time of the trial, the UK National guideline for therapy of Behçet’s indicated that either the tumour necrosis factor alpha inhibitor infliximab or the interferon alpha drug Roferon could be employed as treatment for patients following failure of first-line treatment with standard immunomodulators. The Bio Behçet’s trial was conceived to exploit the opportunity of the three UK National Centres of Excellence for Behçet’s and associated satellite centres to undertake the first randomised clinical trial to compare infliximab and Roferon as treatment for BS, together with an exploratory analysis of potential genomic and metabolomic biomarkers of therapeutic response. Methods The Bio Behçet’s trial is a pragmatic, standard of care, single-masked, randomised, two-arm, parallel trial comparing the efficacies of infliximab and Roferon employed after failure of first-line therapy in BS. Patients with BS, diagnosed according to the International Study Group 1990 criteria, with active disease who had failed to respond to, or were intolerant of, first-line treatment of BS with topical steroids or small-molecule immunosuppressants were randomised to treatment with either infliximab (Remicade) 5 mg/kg intravenous infusions or Roferon subcutaneous injection (in variable dose), utilising the treatment protocol for each of these drugs in normal clinical care as detailed in the BS drugs pathway for England. The trial utilised a Bayesian design utilising priors informed by a small survey of international experts in BS. Utilising a Bayesian analysis of covariance model, with an 80% credible interval, a sample size of 45 patients per arm was deemed appropriate and gave a Bayesian power of 90%. Allowing for an anticipated 10% dropout rate, 100 patients were planned to be recruited but reduced to 80 following recommendations to reduce the overall length of the trial. Allowing for a 10% dropout rate, estimates of study power based on 72 evaluable patients (36 on each arm) and an 80% credible interval a Bayesian power of 88% was obtained. Between June 2016 and February 2020, 161 patients were screened, and 79 patients were randomised. The intention-to-treat analysis was restricted to 37 subjects allocated to infliximab and 37 to Roferon. Based on previous work with hepatitis C infection and response to interferon therapy and given the role of the innate immune system in the pathogenesis of BS, we examined interferon lambda 3 (IFNL3) and interferon lambda 4 (IFNL4) single nucleotide polymorphisms (SNPs) as biomarkers of response to treatment with alpha interferon and/or infliximab in BS. We also examined the potential for urine metabolomics to act as biomarkers for drug response. The primary outcome was a modified version of the Behçet’s disease activity index (mBDAI) after 3 months of therapy. Secondary outcomes comprised mBDAI score after 6 months of therapy and significant improvement in organ systems after 3 and 6 months (week 12 and week 24 visits) assessed by: reduction in vitreous haze using the SUN consensus group grading scale and best corrected visual acuity change [using the logarithm of the minimal angle of resolution (LogMAR) chart at 4 m] from baseline; change in oral ulcer severity score; change in number of genital ulcers; arthritis pain (10 cm Likert scale); adverse events (AEs) in each group; reduction in dose of prednisolone (or equivalent glucocorticoid) at 3 months (week 12 visit); reduction in dose of prednisolone (or equivalent glucocorticoid) at 6 months (week 24 visit); quality-of-life (QoL) scores at 3 and 6 months (week 12 and week 24 visits) and Physician’s Global Assessment of disease activity at 3 and 6 months (week 12 and week 24 visits). Results Baseline characteristics of the two treatment arms did not differ significantly by sex, ethnic profile, baseline disease characteristics and steroid use. For the primary outcome measure, change in mBDAI between baseline and 3 months (and as a secondary outcome between baseline and 6 months) did not differ significantly between the two treatments [mean difference (80% CrI) = 0.13 (–0.19 to 0.46)]. A significantly higher proportion of patients randomised to Roferon swapped away from their randomised treatment compared to those randomised to infliximab treatment (Roferon 11 of 37, infliximab 3 of 37; p = 0.0104). The clinician’s overall perception of disease activity indicated a reduction in disease activity for most patients between baseline and 3 months and between baseline and 6 months, with a median reduction of −2.0 (infliximab) and −1.0 (Roferon) at 3 months and −3.0 (infliximab) and −2.0 (alpha interferon) at 6 months. There was a small but significant difference in favour of infliximab compared to Roferon at both 3 and 6 months (p

The article discusses a study which investigated the risk for pulmonary hypertension (PH) in Behçet's disease patients with Budd-Chiari syndrome. Topics discussed include the clinical characteristics of Behçet's disease patients with BCS, the results of transthoracic echocardiogram, and possible causes of PH in Behçet's disease.

Introduction/ObjectiveMethodsResultsConclusionThere 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.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.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.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]

Objective To explore the correlation between neutrophil-to-lymphocyte ratio (NLR) and Behcet’s disease (BD) activity. Methods A total of 103 BD patients were divided into the low activity group (0-4, 61 cases) and the high activity group (5-11, 42 cases) according to electronic medical record-based disease activity index (EMRAI) score. The white blood cell (WBC), neutrophil (NEU), lymphocyte (LY), platelet (PLT), red blood cell (RBC), hemoglobin (Hb), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), IgG, IgA, IgM, complement C3 and C4 were detected. NLR and platelet-to-lymphocyte ratio (PLR) were calculated. The correlation between NLR, PLR and ESR, CRP, EMRAI were analyzed. Logistic regression was used to analyze the influencing factors of BD disease activity. Receiver operating characteristic (ROC) curve was drawn to evaluate the effectiveness of NLR in judging BD disease activity. Results WBC, NEU, PLT, ESR, CRP, NLR, PLR, complement C3 and C4 in patients were higher in the high activity group than those in the low activity group (P＜0.05), and there were no significant differences in other indexes (P＞0.05). NLR was positively correlated with ESR, CRP and EMRAI in the whole group, while PLR was positively correlated with ESR, CRP and EMRAI in the whole group (P＜0.05). Logistic regression analysis showed that high NLR was a risk factor for BD disease activity (OR=1.511, 95%CI: 1.080-2.113, P＜0.05). ROC curve analysis showed that the area under the curve (AUC) of NLR in evaluating BD disease activity was 0.706 (95%CI: 0.603-0.809). Conclusion NLR is effective in judging the disease activity of BD patients, and can be used as a biological index to evaluate the disease activity of BD. [ABSTRACT FROM AUTHOR]