Behçet's Disease Patients Research Articles

Clinical characteristics and quality of life of patients with Behcet’s disease with arthritis in Japan

Arthritis is one of the most common symptoms of Behcet’s Disease (BD) observed in 57% of Japanese patients with BD. The relationship between arthritis and other clinical symptoms of BD and the impact of arthritis on the quality of life (QOL) of patients with BD are still unclear. Therefore, the current study aimed to clarify the differences in clinical symptoms depending on the presence or absence of arthritis and evaluate the impact of arthritis on QOL in these patients. Fifty-three Japanese patients diagnosed with BD and being treated for more than 6 months were included in this study. Patients were divided based on the presence of arthritis symptoms into an arthritis and a non-arthritis group. Clinical symptoms, disease activity, and QOL of both groups were compared using the Patient’s global assessment (PGA), Evaluator’s global assessment (EGA), and Behcet‘s disease current activity form (BDCAF) as indices of disease activity. To evaluate disease activity related to lesions other than arthritis, BDCAF excluding arthritis items was also calculated. The Behcet’s disease quality of life (BDQOL) scale was used to assess patients’ QOL. Oral ulcers and skin lesions were significantly more frequent in the arthritis group than in the non-arthritis group (p = 0.009 and 0.048, respectively). Among skin domains, papulopustular lesions tended to be more frequent in the arthritis group. EGA and BDCAF (both including and excluding arthritis-related items) scores were significantly higher in the arthritis group (p = 0.019, < 0.0001, and 0.0004 respectively). Although PGA and BDQOL tended to be higher in the arthritis group, the difference between the two groups was not statistically significant. The disease course in Japanese BD patients with arthritis was more frequently complicated by oral ulcers and skin lesions than in those without arthritis. In addition, BD patients with arthritis tended to have generally higher disease activity and low QOL than their non-arthritis counterparts. Further research is required to confirm these results.

Clinical course of pediatric-onset Behçet's Disease in young adulthood.

Although Behçet's disease (BD) typically manifests in the second or third decade of life, initial symptoms may appear at a younger age. It may also take a longer time for the full disease phenotype to develop after the first symptom onset in pediatric patients. In this study we aimed to assess the clinical course of pediatric-onset BD in adulthood period. The files of 112 patients diagnosed with BD before the age of 18, selected from five tertiary clinics, were retrospectively examined. Patients with a follow-up of less than six months were excluded. The study comprised 93 patients with pediatric-onset BD, of whom 64.5% (n = 60) were male. The median age of diagnosis was 15 years (13-17). Major organ involvement was present in 49 (52.5%) patients. The most commonly affected organ was the eye (29%).Sixty-eight (73.1%) patients had follow-up data in adulthood. Fourty patients had only mucocutaneous manifestations in the pediatric period. During follow-up in adulthood, 15 (53.3% were male) had new major organ involvement with a mean of 10.1 (SD: 7.9) years after diagnosis. Twenty-eight patients (41.1%) experienced major organ involvement during the pediatric period. In adulthood follow-up, 12 (42.8%) developed new major organ involvement and/or relapse of the same organ. Eighteen (26.5%) of 68 pediatric-onset BD patients had new major organ involvement, and 9 (13.2%) had a relapse during adulthood follow-up. Our results showed that nearly one-third of pediatric BD patients have a new major organ involvement or a relapse in adulthood. Regular follow-up of pediatric BD patients in adulthood is essential to prevent long-term damage in this disease subset.

A proliferation-inducing ligand (APRIL) level among risk factors of ocular involvement in patients with Behçet’s disease

IntroductionOcular involvement is quite common in Behçet’s disease (BD) and may cause crucial functional complications. Even though the mechanisms of BD remain unclear, advances in gene­tic and immunological fields have improved our understanding of the immunopathogenesis of BD ocular involvement. Little is known about the expression of a proliferation-inducing ligand (APRIL) in terms of ocular involvement in BD. The objective of this study was to determine whether serum APRIL concentrations are associated with ocular involvement in patients with BD.Material and methodsThe study included 60 patients diagnosed with BD matched by age and sex to 30 healthy individuals. Every patient underwent a clinical evaluation, and the Behçet’s Disease Current Activity Form (BDCAF) was utilized to quantify disease activity. All patients underwent a comprehensive ophthalmological assessment. Serum APRIL was assessed for patients as well as controls.ResultsOne or more ocular manifestations were found in 42 BD patients (70%), while 18 patients (30%) had no ocular involvement. The mean level of serum APRIL levels was markedly elevated in BD patients, particularly those with ocular involvement, compared to both BD patients without ocular involvement and healthy individuals. A statistically significant association was determined between high APRIL concentration and development of uveitis, cataract, and hypopyon. Cutaneous lesions and arthritis were strong independent predictors for ocular involvement in BD patients.ConclusionsOverexpression of APRIL in patients with BD, particularly in terms of uveitis, cataract, and hypopyon, lends credence to the idea that B cell activating factors have an important function in BD. These findings may indicate that serum APRIL concentrations can differentiate a clinical subgroup of BD patients with ocular disease. As a result, finding a new therapeutic strategy targeting the APRIL pathway might be beneficial in BD patients suffering from ocular involvement.

Expression Levels of lncRNA NEAT1, miRNA-21, and IL-17 in a Group of Egyptian Patients with Behçet's Disease: Relation to Disease Manifestations and Activity.

Long noncoding ribonucleic acids (lncRNAs), small noncoding RNAs known as microRNAs (miRNAs) as well as some cytokines are recently thought to have a role in many inflammatory and autoimmune disorders including Behçet's disease (BD). This chronic multisystem disease lacks the particular histological or laboratory findings that might aid in its diagnosis. Therefore, any association with such molecules may have an impact on understanding the disease pathogenesis and/or management. The current study compared the levels of NEAT1, miR-21 and IL17 levels in sera of Egyptian BD patients and healthy individuals. The expression levels of these molecules were further investigated for their association with BD manifestations and activity aiming to explore their potential application in disease management. NEAT1 & miR-21 showed down-regulation while IL-17 showed up-regulation among BD patients as compared to controls. IL-17had significant correlation with major vessels involvement and cyclophosphamide intake. NEAT1 showed a significant negative correlation with colchicine intake. Disease activity did not correlate significantly with any of NEAT1, miR-21 or IL-17. NEAT1, miR-21 and IL17 might have a role in Behçet's disease pathogenesis, so more research is needed to unveil that role and their potential usage as biomarkers for the diagnosis or therapeutic targets.

Influence of gender on Behçet's disease phenotype and irreversible organ damage: Data from the International AIDA Network Behçet's Disease Registry

ObjectivesGender impact on phenotypical expression of Behçet's disease (BD) has been specifically investigated only in a few large-scale studies. The main goal of the study was to examine gender differences in a large cohort of patients affected by BD. MethodsData were retrieved from the International AIDA Network Registry for BD. We assessed differences between males and females in terms of Behçet's syndrome Overall Damage Index (BODI), differences in the disease manifestations at onset and in the cumulative manifestations throughout disease course, as well as differences in the cardiovascular risk. Finally, predictive factors leading to major organ involvement were investigated. ResultsIn total, 1024 BD patients (567 males, 457 females) were enrolled in the study, with a male-to-female ratio of 1.24/1. Males displayed a significantly higher mean±SD BODI (1.92±2.09) at the last follow-up, compared to female patients (1.25±1.87) (P<0.0001). Uveitis (P<0.0001) and vascular involvement (P=0.0076) were significantly more frequent among males whereas female patients were significantly over-represented in arthralgia (P<0.0001), arthritis (P=0.00025), isolated headache (P<0.0001), central nervous system (CNS) involvement (P=0.040), and gastrointestinal involvement (P=0.00046). Regarding cardiovascular risk, no differences between the two groups emerged (P=0.617). Four variables were associated with the development of major organ involvement: male gender (OR=2.104, P=0.001), current treatment with biologic agents (OR=2.257, P=0.0003), origin from endemic countries (OR=2.661, P=0.0009), and disease duration (OR=1.002, P=0.024). ConclusionBD displays a more severe course among males. This subgroup develops more irreversible damage and presents more frequently ocular and vascular involvement during disease course. On the other hand, female patients are prone to experience articular involvement, headache, CNS and gastrointestinal involvement. These data suggest the existence of a gender-driven disease expression.

Characterization of potential salivary biomarkers in Behçet's disease.

Diagnosis of Behçet's disease (BD) has always been a challenging task due to the heterogeneity and complexity of the disease, while available literature on BD biomarkers is largely inconclusive. The aim of this study was to evaluate the expression of a panel of salivary biomarkers in a group of BD patients and test their association with BD. The study population comprised 23 BD patients (12 females and 11 males) and a control group of age and gender-matched healthy individuals. Enzyme-linked immunosorbent assays were used to test the expression of 5 salivary proteins including: interleukin 1β (IL-1β), epidermal growth factor (EGF), endothelin, vascular endothelial growth factor (VEGF) and transferrin (TrF). BD activity was evaluated using the International Society for Behçet's disease (ISBD) current activity form. Salivary endothelin, EGF and VEGF were significantly higher in the BD group compared to controls (16.3 Vs. 7.7pg/ml, 194.5 Vs. 163.4pg/ml and 262 Vs. 150.2pg/ml, p<0.05, respectively) whereas TrF was significantly lower in BD group compared to controls (497 Vs. 2206pg/ml, p<0.001). The correlation analysis showed a trend of a positive linear association between endothelin expression and both BD activity (r=0.41) and BD activity index score (r=0.4), without reaching statistical significance (p>0.05). In conclusion, salivary TrF, endothelin, VEGF and EGF may represent potential biomarkers for BD. These findings require further validation in larger samples and different activity states of BD.

Postoperative complications in patients with Behçet’s disease

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

Anticoagulant Treatment May Decrease the Relapse Rate of Pulmonary Arterial Involvement in Behçet's Disease.

Pulmonary arterial involvement (PAI) is one of the most common causes of mortality in Behçet's disease (BD). In this study, we aimed to evaluate the clinical features, course, and recurrence risk factors of BD-associated PAI. BD patients who were followed up in Marmara University BD outpatient clinic between 1990 and 2023 were included. All data were acquired from the medical records of the patients. PAIs were classified according to the type of the vascular involvement as thrombosis or aneurysm. Factors affecting the risk of relapses were determined using multivariate Cox regression analysis. Among 1350 BD patients, 110 (8.1%) had PAI. The mean age (SD) of patients with PAI was 42.4 (11.6) years, and the male/female ratio was 2.2 (76/34). Thirty-two (29.1%) of 110 patients were asymptomatic. Asymptomatic patients with PAI were significantly younger (p = 0.031) than the symptomatic group. Also, a greater prevalence of females (p = 0.001) and higher recurrence rates (p = 0.019) were observed in the symptomatic group. Pulmonary arterial thrombosis was seen in 104 (94.5%) and aneurysms in 9 patients (6.6%). At least one PAI relapse was observed in 31 patients (28.2%). In multivariate analysis, the Cox regression model was significant (p = 0.013), and not starting anticoagulants independently increased the PAI relapse risk (hazards ratio, 4.36; 95% confidence interval, 1.14-24.1; p = 0.042). Pulmonary arterial thrombosis is the main presentation type of PAI in BD, whereas aneurysmatic formation is rare. Despite immunosuppressive treatment, relapses occur during follow-up in one third of patients with PAI. When anticoagulant therapy is added to immunosuppressive therapy, the relapse rate in BD patients with PAI is significantly reduced.

ABO blood groups and increased risk for the development of vascular involvement in Behçet's disease.

Inflammatory endothelial activation is a prominent feature of vascular involvement in Behçet's disease (BD), which is usually accompanied by a thrombotic tendency. Recent studies suggest an association between ABO blood groups and thrombotic vascular disease in those carrying non-O (A, B, and AB) groups. This study aimed to analyse the contribution of ABO blood groups to the vascular involvement in BD. In this retrospective study, BD patients with available ABO blood group data and meeting the ISG criteria were included in the study. The presence of vascular involvement, its clinical characteristics, and the data about other manifestations were recorded from the patient charts. This study was conducted in 411 patients; 143 (34.8%) were carrying O and 268 (65.2%) were carrying non-O groups. The vascular involvement was significantly more frequent and the risk for overall vascular and venous events was up to 2-fold higher in the patients with non-O groups. After adjustments for age, sex, and comorbidities, the risk for arterial disease was also found to be increased in association with non-O groups. ABO blood groups were shown to be independent risk factors for vascular BD by multiple regression models based on known predisposing factors. Compared with other non-O blood groups, the patients carrying blood group B had a higher risk for vascular events. The results of this preliminary study show the potential contribution of ABO blood groups to the vascular-BD phenotype and suggest an increased risk for vascular BD in association with non-O blood groups.

The burden of mental health issues in Behçet’s disease: implications for patient quality of life

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

Evaluation of epicardial adipose tissue thickness and carotid intima-media thickness in children with Behçet's disease.

Behçet's disease (BD) is a systemic vasculitis affecting all sizes of arteries and veins. Approximately 5-10% of patients with BD are present during childhood. The chronic nature of the disease may lead to serious cardiovascular complications over time including early atherosclerosis. Increased levels of epicardial adipose tissue (EAT) and carotid intima-media thickness (CIMT) are considered early signs of subclinical atherosclerosis. Ongoing chronic inflammation may cause to increase in both EAT and CIMT. In this study, we aimed to evaluate CIMT and EAT in children with BD and determine their relationship with the clinical manifestations and course of the disease. This cross-sectional study evaluated 30 patients with juvenile-onset BD and age-sex-matched 20 healthy controls. The CIMT and EAT thickness were measured by the same paediatric cardiologist. The association between clinical features, baseline disease activity, disease duration, EAT thickness and CIMT was also evaluated. Thirty children with BD and 20 age-sex-matched healthy volunteers enrolled in the study. The most common BD-related feature was oral aphthous (n=30), followed by mucocutaneous findings (n=22). Uveitis was observed in 5 patients, vascular involvement in 4, neurological involvement in 4, and gastrointestinal involvement in 2. All patients were inactive at the time of evaluation. The EAT thickness was significantly higher in patients while CIMT levels revealed no significant differences. However, there was no correlation between disease duration, baseline disease activity, and EAT thickness. Increased EAT thickness may be a risk factor for early atherosclerosis in patients with BD. The EAT thickness was found to be significantly higher in paediatric BD patients. Confirmation of results in larger series may provide better insight into early screening for risk factors in these patients.

Role of Optical Coherence Tomography in Vasculitis-Associated Pulmonary Hypertension and Chronic Thromboembolic Pulmonary Hypertension.

Identifying and understanding the microstructural changes within the wall of the pulmonary artery (PA) is crucial for elucidating disease mechanisms and guiding treatment strategies. We assessed the utility of optical coherence tomography (OCT) in identifying such changes within segmental/subsegmental PAs and compared the morphological variations in WHO group 4 pulmonary hypertension associated with Behcet Disease (BD), Takayasu arteritis (TA) and chronic thromboembolic pulmonary hypertension (CTEPH). Idiopathic pulmonary arterial hypertension (IPAH) patients served as controls.Methods and Results: A total of 197 cross-sectional images were analyzed from 20 consecutive patients. BD patients exhibited lower %wall area and mean wall thickness (MWT) compared with CTEPH, TA and, IPAH patients. TA patients showed a notably higher %wall area, which was significant in IPAH and BD patients. Variations in %wall area measurements were observed across distinct cross-sectional segments of the PA within individual patients (22% in CTEPH, 19% in BD, 16% in TA, 23% in IPAH patients). Intravascular webs, bands, and thrombi were observed in BD and CTEPH patients. OCT provided clear delineation of vascular wall calcifications and adventitial vasa vasorum. No procedure-related complications were observed. PA involvement differs among the various etiologies of PH, with the PA being heterogeneously affected. OCT offers promise in elucidating microstructural vascular wall changes and providing insights into disease mechanisms and treatment effects.

Protein tyrosine phosphatase non-receptor type 2 (PTPN2) gene polymorphisms (rs2542151, rs7234029) in Egyptian Behçet’s disease patients: a preliminary report

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.

Regulatory role of the lncRNAs MIAT and PVT1 in Behçet’s disease through targeting miR-93-5p and miR-124-3p

BackgroundNoncoding 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.MethodsBioinformatic 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.ResultsThe 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.ConclusionMechanistically, 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)

Behçet's disease modifies the gingival inflammatory response.

Behçet's disease (BD) pathogenesis involves severe outcomes such as blindness, central nervous system manifestations, and deep venous thrombosis that impacts systemic and local inflammatory changes. We tested the hypothesis that BD negatively affects gingival health and increases the severity of gingivitis. The study included 37 BD patients with gingivitis without any sign of periodontitis. Systemically healthy 19 patients with gingivitis (G) and 20 periodontally and systemically healthy individuals (C) were recruited as controls. BD patients were further grouped as stable and unstable based on their responses to BD treatment. Clinical periodontal parameters were measured to determine the impact of BD on gingival health. Serum and saliva levels of ELA-2 (neutrophil elastase-2), SLPI (secretory leukocyte protease inhibitor), α1-AT (alpha1-anti-trypsin), VEGF (vascular endothelial growth factor), IL-6 (interleukin-6), IL-8 (interleukin-8), and TNF-α (tumor necrosis factor alpha) were analyzed using multiplex immunoassay to measure the systemic and local inflammatory impact of BD. Plaque index (PI), probing pocket depth (PPD), and bleeding on probing (BOP) were significantly higher in the BD group than in the controls (p<0.05). IL-6 was higher in both serum and saliva in the BD group than in the G group (p<0.05). ELA-2 levels in saliva were higher in the stable BD group than in the controls, while TNF-α and SLPI were statistically significantly higher in BD than in the control (p<0.05). Salivary α1-AT level was statistically lower in the BD group compared to the control group. Our study suggested that the gingival inflammatory profile was impaired in patients with BD.

Clinical Features of Behçet’s Disease Uveitis

Abstract: Behçet’s disease (BD) is a chronic, multisystemic vasculitis first described by Professor Hulusi Behçet in 1937. It is characterized by recurrent inflammatory attacks affecting multiple organs, with uveitis being a significant and severe complication that can lead to blindness. BD typically emerges between the ages of 20 and 30 years, with a higher prevalence in males and rare occurrences in children. The prevalence and severity of BD and its ocular symptoms tend to diminish with age. In Turkey, BD is a leading cause of noninfectious uveitis, and its prevalence varies across different regions. Ocular involvement, seen in up to 90% of BD patients, often appears 2–4 years after disease onset and may be the initial sign in 10%–20% of cases. BD uveitis is characterized by recurrent nongranulomatous panuveitis and retinal vasculitis, typically affecting both eyes. Diagnostic tools such as fluorescein angiography (FA), optical coherence tomography (OCT), and laser flare photometry (LFP) are essential for monitoring disease activity and guiding treatment. FA is crucial for identifying occlusive and leaky vasculitis, while OCT helps in detecting macular complications and visualizing retinal infiltrates and their sequelae. LFP quantitatively evaluates intraocular inflammation. Recognizing the ocular manifestations of BD early is vital for accurate diagnosis and effective management. This review highlights the clinical features, diagnostic tools, and importance of early diagnosis in managing BD uveitis.

Evaluation of right ventricular function in patients with Behcet's disease by four-dimensional echocardiography.

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). 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. 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. 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.

Characteristics of late-onset Behçet's disease and comparison with juvenile and adult-onset Behçet's disease.

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

Diffusion tensor imaging in Behcet's disease with and without neurological involvement patients: evaluation of microstructural white matter abnormality with a tract-based spatial statistical analysis.

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. 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. 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. 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. 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.

Clinical profiling, treatment characteristics and outcome in Behcet's Disease (BD)-A retrospective cohort study from Karnataka Rheumatology Association (KRA).

Behcet's disease (BD) has a heterogeneous and unpredictable phenotype that differs in various geographical areas. To describe the clinical phenotype & outcome of Behcet's disease(BD) from Karnataka, India and compare them with large cohorts from endemic regions. 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. We included 72 patients, equal gender distribution and mean age 37.4 ± 12.8years 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. 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.