Nailfold videocapillaroscopy abnormalities and vascular manifestations in Behçet's syndrome.

OBJECTIVES: To evaluate microcirculation abnormalities and their clinical association in patients with BS, especially with vascular manifestations. METHODS: A cross-sectional study was carried out using nailfold videocapillaroscopy (NVC) to evaluate the microcirculation in patients with BS. RESULTS: A total of 65 patients were included in the study. Thirty-four (52.3%) were men, and 84.6% were European Caucasian. Vascular involvement was present in 24 (36.9%) patients. Qualitative abnormalities in NVC were observed in 47.7% of patients. The most frequent were tortuous and branched capillaries (21.5%), followed by microhaemorrhage (12.3%), enlarged capillary (7.7%) and giant capillary (3.1%). We found a significant relationship between the presence of tortuous and branched capillaries and previous superficial thrombophlebitis (ST) (p=0.025). The presence of ≥2 qualitative abnormalities in NVC was associated with vascular involvement (p=0.031), mainly with venous thrombotic events (p=0.024) and particularly with ST (p=0.003). No specific Cutolo's pattern was observed. No association was observed between NVC and Raynaud's phenomenon or ANA, although patients with positive ANA presented more frequent non-specific capillaroscopic abnormalities (p=0.003). CONCLUSIONS: The presence of ≥2 qualitative abnormalities in NVC is associated with Vasculo-Behçet. NVC might be a potential tool for early detection of patients at risk of vascular events.

Sensitive optical coherence tomography angiography parameters detecting retinal vascular changes in Behcet's uveitis.

PURPOSE: To compare the retinal parameters in Behcet's uveitis (BU) patients with wide-field swept-source optical coherence tomography angiography (SS-OCTA) and find a sensitive OCTA parameter. METHODS: Fifty-two eyes from 52 quiescent BU patients and 50 healthy eyes were included. All subjects underwent SS-OCTA examinations with 12 × 12 mm region. Vessel density (VD) and flow area (FA) in nerve fiber layer (NFL), superficial vascular plexus (SVP), intermediate capillary plexus (ICP) and deep capillary plexus (DCP) were analyzed and compared in central, parafoveal, and peripheral regions with diameters of 1, 6 and 12 mm. Receiver operating characteristic curves (ROC), area under the curve (AUC), correlation analysis between OCTA metrics and best-corrected visual acuity (BCVA) were respectively evaluated. RESULTS: BU patients showed significantly lower peripheral VD and FA in NFL (P = 0.019 and 0.002), lower central and parafoveal VD-SVP (P = 0.006 and <0.001), and lower VD-ICP, VD-DCP, FA-SVP, FA-ICP and FA-DCP in all regions (all P < 0.05) as compared to healthy controls. The ROC analysis indicated that the parafoveal, peripheral FA-DCP-1, and a combination of the two metrics were sensitive parameters for identifying retinal vessel changes in BU (AUC=0.90, 0.90, 0.91, respectively). The parafoveal and peripheral FA-DCP were negatively associated with logMAR BCVA (r=-0.764, P < 0.0001; r=-0.641, P < 0.0001). CONCLUSION: The deep retinal layers were apt to be affected in BU patients. The parafoveal and peripheral FA values of DCP may be sensitive parameters for detecting retinal vasculature alterations in BU.

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

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.

Parenchymal Neuro-Behçet's disease or Comorbid Behçet's disease with multiple sclerosis: A discriminative analysis of a complex clinical entity.

BACKGROUND: Patients with Behçet's disease (BD) may rarely manifest with cerebral white matter lesions resembling multiple sclerosis (MS). This may result in misdiagnosis due to diagnostic difficulties between parenchymal neuro-BD (pNBD) and MS. This study aims to elucidate the distinguishing features of patients with comorbid BD and MS (BD+MS) in comparison to those with pNBD and MS alone by focusing on clinical and laboratory features. We also aimed to identify the distinctive characteristics of BD+MS patients by comparing them to patients with pNBD and MS. METHODS: The methodology of this study involved a retrospective analysis of patient records followed in the Department of Neurology at the Istanbul Faculty of Medicine, Istanbul University. The study population included patients diagnosed with pNBD, MS, and a comorbid condition of BD and MS (BD+MS). We assessed clinical, radiological, and laboratory data, including disease onset, annual relapse rates, Expanded Disability Status Scale (EDSS) progression, and cerebrospinal fluid examination. Several parameters were examined between the pNBD, MS, and BD+MS patient groups to find similarities and differences between subgroups. RESULTS: Our study included 1,764 patients: 172 with pNBD, 1,574 with MS, and 18 with BD+MS. A predominance of females was noted in the BD+MS (72%, p < 0.001) and MS (69 %, p < 0.001) groups compared to pNBD (30 %). The median age at the onset of neurological symptoms was 35.5 (IQR: 16.8) years for BD+MS, 34.6 (13.6) years for pNBD, and 27.6 (13.3) years for MS (BD+MS vs. MS; p = 0.3, pNBD vs. MS, p = 0.7). Additionally, the number of attacks was notably different, with BD+MS patients experiencing a median of 3.5 (2.0) attacks compared to 3.0 (3.0) for MS patients and only 1.0 (1.0) for pNBD patients, suggesting a more active disease course in the MS and BD+MS groups compared to pNBD (p < 0.001). The median annualized relapse rate for BD+MS was 0.3 (0.2), which was lower than the rate of 0.4 (0.4) in MS (p = 0.048) and equivalent to the rate of 0.2 (0.3) in pNBD (p = 0.2). The time to the first relapse was similar to those with BD+MS and MS, but considerably shorter than in individuals with pNBD (p < 0.0001). The cerebrospinal fluid (CSF) analysis showed no significant differences in neutrophil and lymphocyte counts between BD+MS and MS patients but elevated levels in pNBD patients (p < 0.05). CSF protein levels were consistent across all groups (p = 0.1 and p = 0.7). Oligoclonal bands were detected in all patients with BD+MS, in the majority of MS patients (83.6 %), and a small percentage of pNBD patients (19.7 %), showing a notable distinction between the BD+MS and pNBD groups (p < 0.001). CONCLUSION: Our study underscores the need for a skeptical approach in diagnosing and treating patients with BD who exhibit symptomatic MS-like MRI lesions. Our findings suggest that BD+MS is a distinct clinical entity, warranting specific diagnostic and treatment approaches. Our findings highlight that BD patients with MS-like lesions meeting MS diagnostic criteria should be managed as patients with comorbid MS and BD rather than pNBD.

Behçet's Disease, Pathogenesis, Clinical Features, and Treatment Approaches: A Comprehensive Review.

Behçet's disease is a systemic inflammatory disorder of unknown etiology. The disease manifests with diverse clinical symptoms, most commonly recurrent oral and genital ulcers, skin lesions, and uveitis, though it can affect multiple organ systems. Diagnosis is primarily clinical due to the lack of a definitive diagnostic test, and management involves a multidisciplinary approach to control inflammation and manage symptoms. Current treatment strategies involve corticosteroids, immunosuppressive agents, and, increasingly, biological therapies. Behçet's disease exhibits a higher prevalence along the Silk Road, suggesting a role of environmental and genetic factors. Despite significant progress in understanding its clinical characteristics and treatment approaches, gaps remain in our understanding of its pathogenesis. Future research is needed to elucidate the disease's pathophysiology and optimize treatment strategies.

Fetal cardiac functional changes in pregnancies with Behcet's disease: A case-control study.

BACKGROUND: Behcet's disease usually progresses with remission during pregnancy, but early subtle changes might be detected in the heart of these fetuses due to inflammation. AIMS: We aimed to evaluate the cardiac functions in fetuses of pregnant women with Behcet's disease (BD). STUDY DESIGN: Prospective case-control study. SUBJECTS: This prospective study enrolled pregnant women diagnosed with Behcet's disease before pregnancy. Twenty-four pregnancies with Behcet's disease and 48 healthy pregnancies were included at 32-34 gestational weeks. OUTCOME MEASURES: Pulsed-wave Doppler and tissue Doppler imaging (TDI) were used to assess cardiac functions. RESULTS: Right ventricle (RV) E (early) wave and left ventricle (LV) E were significantly increased in pregnancies with BD (p = .008, p = .041, respectively). Decreased right ventricle E' (peak systolic velocity) was detected with TDI in the case group (6.2 ± 0.5, p < .001). E/E' ratios for RV and LV were significantly increased in the case group (p < .001, p = .001, respectively). The correlation between the duration of the disease and fetal cardiac functions was also evaluated. For RV, E (r = 0.735, p < .001), E' (r = -0.735, p < .001), E/E' (r = 0.894, p < .001), were strongly correlated with the disease duration. The study also showed the correlation between disease duration and LV E' (r = -0.735, p = .005), LV E (r = 0.750, p < .001), and LV E/E' (r = 0.820, p < .001). CONCLUSION: This is the first study to evaluate the fetal cardiac functions in fetuses of pregnancies with BD. Although BD usually progresses with remission during pregnancy, early subclinical diastolic changes might occur in the heart of these fetuses due to inflammation.

Treatment Adherence Effect on the Visual Acuity of Behçet's Uveitis Patients.

PURPOSE: This study aimed to explore the relationship between treatment adherence, visual acuity, quality of life (QoL), depression, and anxiety levels in individuals with Behçet's uveitis (BU). METHODS: A total of 55 BU patients and 55 healthy controls completed sociodemographic questionnaires, the Beck Depression Inventory (BDI), Beck Anxiety Inventory (BAI), World Health Organization Quality of Life Questionnaire-BREF version (WHOQOL-BREF), Self-reported Questionnaire About Treatment Compliance, and the Morisky Medication Adherence Scale (MMAS). The scores obtained from these scales were analyzed to assess the relationship between treatment adherence, visual acuity, QoL, depression, and anxiety levels. RESULTS: The results revealed that 36 (65.4%) of the 55 BU patients exhibited depressive symptoms, while 45 (81.8%) of them experienced symptoms of anxiety. Patients with BU demonstrated significantly higher BAI and BDI scores compared to the healthy control group (p < 0.001). Furthermore, the patient group reported lower mean scores across multiple domains of the WHOQOL-BREF questionnaire, including general health, psychological health, social relationships, and environment (p < 0.05). Moreover, a significant association was observed between low treatment adherence and lower values of best-corrected visual acuity (BCVA) (p < 0.05), as well as a higher frequency of uveitis attacks (p = 0.005). CONCLUSION: Poor treatment adherence in BU patients has a negative effect on final visual acuity outcomes. Moreover, BU patients experience lower QoL and higher rates of depression and anxiety compared to the healthy control group. These findings highlight the importance of addressing treatment adherence and psychological well-being in the management of BU.

Validity and reliability of a checklist for patients with Behçet's disease based on the International Classification of Functioning, Disability and Health.

In 2020, we reported the "BD-checklist 92" for patients with Behçet's disease (BD) based on the International Classification of Functioning, Disability and Health. The purpose of the present study was to evaluate the validity and reliability of this checklist. Questionnaires using the "BD-checklist 92" and the 36-item Short Form Survey (SF-36) were sent to ten affiliated institutions. In total, 174 patients answered the questionnaire (response rate, 32.7%). Criterion validity was evaluated using the correlation coefficient between the number of problem categories extracted from the "BD-checklist 92" and the scores of the eight subscales and two components of the SF-36. Construct validity was assessed based on the number of problem categories extracted as an external criterion for the number of manifestations experienced and specific lesions. The comparison was performed using the Mann-Whitney U test. Cronbach's alpha coefficient was used to evaluate reliability. The number of problem categories in the "Body functions and structures", "Activities and participation", and "Environmental factors" components correlated significantly with all dimensions of the SF-36 questionnaire (P < 0.05 each). Construct validity showed that the number of manifestations experienced in all components (P < 0.001 each) and specific lesions in "Body functions and structures" and "All categories" (P = 0.002 and 0.050, respectively) contributed to an increased number of problems associated with BD. Cronbach's alpha coefficient for the "BD-checklist 92" was 0.926. This study confirmed the validity and reliability of the "BD-checklist 92".

Sexual dysfunction and depression in Behçet's disease in comparison to healthy controls.

Behçet's disease (BD) can affect the genital system and is more common in Middle Eastern countries and Asia but also occurs in Caucasian people. Aim of this study was to evaluate the prevalence of sexual dysfunction (SD) and depression in patients with BD compared to a healthy control group (HCG). In addition, differences with regard to depression and patients' origin were evaluated. This prospective, monocentric study included 106 consecutive patients from our specialized BD outpatient clinic. Patients were asked to fill out the paper based standardized and validated questionnaires International Index of Erectile Function (IIEF), the Female Sexual Function Index (FSFI) and the Beck Depression Inventory (BDI). In addition, 206 healthy controls were asked to fill out the questionnaires. 106 patients with BD were evaluated and 206 participants in the HCG. The mean age in BD group was 40.5 years as compared to 44.4 years in the HCG. Half of the patients had Middle Eastern and half Caucasian origin. SD was found in 24.5% of all subjects. Only 6.9% of male patients showed signs of SD, while half of the women's group was suffering from SD. The prevalence for SD was significantly higher in women with Middle Eastern ethnic origin compared to women with Caucasian origin (75 vs. 33.3%, p = 0.024). Erectile Dysfunction occurred in 55% of all male patients which was not statistical different from the HCG. Genital ulcers affected 73.6% of all patients. Depression was found in 36.7% of all subjects as compared to 6.25% in the HCG (p < 0.001). Both, SD and depression correlated positively in males (p = 0.017) and females (p = 0.013). SD and depression are very common problems in BD and should be addressed by the treating physician. Both manifestations are intensifying each other. Depression especially is more prevalent compared to the healthy population.

Behçet's disease and activities of daily living.

OBJECTIVES: No large-scale registration study has comprehensively evaluated the activities of daily living (ADL) in patients with Behçet's disease. METHODS: The Japanese government provided us with a dataset of confirmed or suspected Behçet's disease cases derived from ongoing national registration. ADL were categorized and analysed into four categories in patients who satisfied the International Criteria for Behçet's Disease. RESULTS: Data from 2960 patients (men 38.9%, women 61.1%; median age 39 years) were assessed. While 1767 patients (59.7%) had normal ADL, the others had impaired ADL comprising limited but not assisted [n = 1058 (35.7%)], partially assisted [n = 116 (3.9%)] and fully assisted [n = 19 (0.6%)]. Logistic regression analysis showed that chronic ocular lesions [odds ratio (OR) 1.85 (95% CI 1.46, 2.35), P < 0.001], paralysis [OR 2.51 (95% CI 1.58, 3.97), P < 0.001], psychosis [OR 3.16 (95% CI 2.02, 4.95), P < 0.001] and arthritis [OR 1.69 (95% CI 1.44, 1.99), P < 0.001] led to the risk of impaired ADL. Chronic ocular lesions [OR 3.61 (95% CI 2.27, 5.72), P < 0.001], paralysis [OR 3.43 (95% CI 1.87, 6.30), P < 0.001] and psychosis [OR 3.60 (95% CI 2.00, 6.50), P < 0.001] were related to the requirement of physical assistance (partially or fully assisted), although arthritis [OR 1.39 (95% CI 0.93, 2.06), P = 0.108] was not a significant factor in this model. CONCLUSION: Ocular lesions, neurological manifestations and arthritis affected ADL. Patients with ocular lesions or neurological manifestations more frequently required physical assistance.

Epidemiological, clinical, and therapeutic characteristics of Behçet's disease: a monocentric study in Tunisia.

INTRODUCTION: to describe the epidemiological, clinical, therapeutic and evolving characteristics of Behçet´s disease and identify prognostic factors. METHODS: we have realized a retrospective, single-center study, conducted over a period of 26 years and including 130 patients presenting Behçet´s disease and hospitalized in an Internal Medicine Department. RESULTS: the mean age of the Behçet´s disease at onset was 30.3 ±8.8 years and that at diagnosis was 34.6 ±9.4 years. The sex ratio (male/female) was 2.5. The mean delay of diagnosis was 53.5 months. Oral aphthosis was constant. The frequency of the manifestations was: genital aphtosis 71.5%, pseudofolliculitis 84.6%, erythema nodosum 11.5%, positive pathergy test 50%, ocular disease 36.9%, venous thrombosis 30%, arterial disease 4.6%, joint damage 30.8%, neurological disease 19.2% and digestive disease 0.8%. The male gender was significantly associated with ocular involvement (p =0.02), venous disease (p =0.01) and occurrence of relapses (p =0.01). The mean follow up was 68.5 ± 77.3 months. The poor survival prognostic factors were male gender, ocular involvement, venous disease, cardiovascular disease, a duration of follow up ≤12 months and a diagnostic delay ≤ 24 months. Conclusion: improving the prognosis of Behçet´s disease requires a shortening of the time to diagnosis, multidisciplinary collaboration, intensive treatment of functional threats, regular monitoring, and patient adherence.

Optical coherence tomography angiography findings in patients with ocular and non-ocular Behcet disease / Achados de angiografia por tomografia de coerência óptica em pacientes com doença de Behçet com e sem acometimento ocular

ABSTRACT Purposes: To evaluate the optical coherence tomography angiography findings in patients with Behçet disease with and without ocular involvement. Methods: A total of 40 patients with Behçet disease and 30 healthy controls were enrolled in the study. Retinal vessel density in the superficial capillary plexus and deep capillary plexus, foveal avascular zone area and perimeter, acirculatory index, foveal density, and nonflow area in the superficial retina were automatically measured using the optical coherence tomography angiography software AngioVue and compared between the groups. Results: The mean parafoveal and perifoveal vessel densities in the superficial capillary plexus and deep capillary plexus and foveal density were significantly lower in the eyes with Behçet uveitis compared to the eyes without Behçet uveitis and eyes of the healthy controls. In the eyes with Behçet uveitis, logMAR visual acuity showed a moderate correlation with parafoveal and perifoveal vessel densities and foveal density (r=-0.43, p=0.006; r=-0.62, p<0.001; r=-0.42, p=0.008; respectively). Conclusion: Behçet disease with posterior uveitis was associated with significant perifoveal and parafoveal vascular decrements in the superficial and deep retina.(AU)

RESUMO Objetivo: Avaliar achados de angiografia por tomografia de coerência óptica em pacientes com doença de Behçet com e sem acometimento ocular. Métodos: Foram incluídos 40 pacientes com doença de Behçet e 30 controles saudáveis. A densidade vascular retiniana nos plexos capilares superficial e profundo, a zona avascular foveal, o índice de circularidade, a densidade foveal e a área sem fluxo da retina superficial foram medidos automaticamente, através do software AngioVue para angiografia por tomografia de coerência óptica, e comparados entre os grupos. Resultados: A densidade vascular parafoveal e perifoveal média nos plexos capilares superficial e profundo, bem como a densidade foveal, foram significativamente menores nos olhos com uveíte de Behçet em comparação com os olhos sem uveíte de Behçet e os olhos dos controles saudáveis. Nos olhos com uveíte de Behçet, a acuidade visual logMAR mostrou correlação moderada com a densidade vascular parafoveal e perifoveal e com a densidade foveal (respectivamente, r=-0,43, p=0,006; r=-0,62, p<0,001; e r=-0,42, p = 0,008). Conclusão: A doença de Behçet com uveíte posterior foi associada a decréscimos significativos da vascularização perifoveal e parafoveal na retina superficial e profunda.(AU)

Genetic and immunologic findings in children with recurrent aphthous stomatitis with systemic inflammation.

BACKGROUND: Recurrent aphthous stomatitis with systemic signs of inflammation can be encountered in inflammatory bowel disease, Behçet's disease (BD), Systemic Lupus Erythematosus (SLE). In addition, it has been proposed that cases with very early onset in childhood can be underpinned by rare monogenic defects of immunity, which may require targeted treatments. Thus, subjects with early onset recurrent aphthous stomatitis receiving a clinical diagnosis of BD-like or SLE-like disease may deserve a further diagnostic workout, including immunologic and genetic investigations. OBJECTIVE: To investigate how an immunologic, genetic and transcriptomics assessment of interferon inflammation may improve diagnosis and care in children with recurrent aphthous stomatitis with systemic inflammation. METHODS: Subjects referred to the pediatric rheumatologist for recurrent aphthous stomatitis associated with signs of systemic inflammation from January 2015 to January 2020 were enrolled in the study and underwent analysis of peripheral lymphocyte subsets, sequencing of a 17-genes panel and measure of interferon score. RESULTS: We enrolled 15 subjects (12 females, median age at disease onset 4 years). The clinical diagnosis was BD in 8, incomplete BD in 5, BD/SLE overlap in 1, SLE in 1. Pathogenic genetic variants were detected in 3 patients, respectively 2 STAT1 gain of function variants in two patients classified as BD/SLE overlap and SLE, and 1 TNFAIP3 mutation (A20 haploinsufficiency) in patients with BD. Moreover 2 likely pathogenic variants were identified in DNASE1L3 and PTPN22, both in patients with incomplete BD. Interferon score was high in the two patients with STAT1 GOF mutations, in the patient with TNFAIP3 mutation, and in 3 genetic-negative subjects. In two patients, the treatment was modified based on genetic results. CONCLUSIONS: Although recurrent aphthous stomatitis associated with systemic inflammation may lead to a clinical diagnosis of BD or SLE, subjects with early disease onset in childhood deserve genetic investigation for rare monogenic disorders. A wider genetic panel may help disclosing the genetic background in the subset of children with increased interferon score, who tested negative in this study.

Clinical profile and evolution of patients with juvenile-onset Behçet's syndrome over a 25-year period: insights from the AIDA network.

Behçet's syndrome (BS) represents an understudied topic in pediatrics: the main aims of our study were to characterize demographic and clinical features of a cohort of BS patients with juvenile-onset managed in three tertiary referral centers in Italy, evaluate their evolution in the long-term, and detect any potential differences with BS patients having an adult-onset. Medical records of 64 juvenile-onset and 332 adult-onset BS followed-up over a 2-year period were retrospectively analyzed and compared. Mean age ± SD of first symptom-appearance was 10.92 ± 4.34 years with a female-to-male ratio of 1.06:1. Mucocutaneous signs were the most frequent initial manifestations, followed by uveitis. Throughout the disease course, genital aphthae (76.56%) and pseudofolliculitis (40.63%) prevailed among the mucocutaneous signs, while major organ involvement was represented by gastrointestinal and ocular involvement (43.75 and 34.38%, respectively). No significant differences emerged for both mucocutaneous signs and specific major organ involvement between juvenile-onset and adult BS patients. After excluding nonspecific abdominal pain, juvenile-onset BS patients were less frequently characterized by the development of major organ involvement (p = 0.027). Logistic regression detected the juvenile-onset as a variable associated with reduced risk of long-term major organ involvement (OR 0.495 [0.263-0.932], p = 0.029). In our cohort, juvenile-onset BS resembled the clinical spectrum of adult-onset patients. Pediatric patients with a full-blown disease at onset showed a more frequent mucocutaneous involvement. In addition, patients with juvenile-onset seemed to develop less frequently major organ involvement and had an overall less severe disease course.

Parafoveal Microvascular Alterations in Ocular and Non-Ocular BehÒ«et's Disease Evaluated With Optical Coherence Tomography Angiography.

Purpose: To compare quantitative optical coherence tomography angiography (OCT-A) measurements of the parafoveal microvasculature in retinal capillary plexuses among BehÒ«et uveitis (BU) patients, non-ocular BehÒ«et's disease (NOBD) patients, and healthy volunteers (HVs). Methods: Sixty-eight subjects were enrolled in this prospective observational cross-sectional study. OCT-A imaging was performed using the Heidelberg Engineering Spectralis OCT. A custom algorithm was developed to calculate the vessel density (VD) in three retinal vascular layers: deep capillary plexus, intermediate capillary plexus, and superficial vascular plexus. The foveal avascular zone (FAZ) and acircularity index were calculated for the whole retinal vascular complex. Results: We analyzed one eye from 21 BU patients (age, 51 ± 10 years), 23 NOBD patients (age, 48 ± 14 years), and 22 HVs (age, 44 ± 13 years). One-way multivariate analysis of covariance showed a statistically significant difference in VD among the three groups when combining the layers after controlling for scan quality (P < 0.001). The VD was lowest in the BU group and highest in the HV group in all layers. The FAZ area was also statistically significant different among the groups (P < 0.005), with the largest FAZ areas in BU patients and smallest FAZ areas in the HV group. However, no statistically significant difference was found for the acircularity index. Conclusions: The parafoveal microvasculature is affected not only in BU patients but also in NOBD patients. Most deviations in the retinal microcirculation in BehÒ«et patients were found in the deeper layers of the retina by using the quantitative VD measurement.

Consistency of Visual Evoked Potential in Extraocular Manifestations of Behcet Disease and Impact of Corticosteroid Treatment.

PURPOSE: To study the ability of pattern reversal visual evoked potentials (VEPs) to detect subclinical visual pathway pathologic assessment in Behcet disease (BD) and correlate VEP results in BD with disease activity and response to corticosteroid treatment. METHODS: This study included 18 patients (36 eyes) with BD without recent ocular manifestations and 20 healthy controls (40 eyes). Clinical history was taken, and clinical examinations were done. Visual evoked potential recordings were performed by an electromyographic evoked machine for patients and controls. Corticosteroids were given to patients with extraocular activity, and VEP was repeated after clinical and laboratory improvement of the manifestations. RESULTS: p100 latency of VEP was prolonged in 8 patients (11 eyes) (44.4% of cases). Mean p100 latency showed statistically significant prolongations in BD group compared with the control group (106.7 ± 8.1 ms vs. 99.6 ± 1.9 ms) with P value < 0.001. There was a positive correlation between p100 latency and the disease activity score (r = 0.8673), whereas there was no correlation between p100 latency and the disease duration (r = 0.00072). Patients treated with corticosteroids showed statistically significant reduction in mean p100 latency after treatment (P < 0.001). CONCLUSIONS: Visual evoked potential may be a valuable tool for detecting subclinical neurologic affection in BD.

Multimodal Imaging Characteristics and Functional Test Findings in a Case of Acute Macular Neuroretinopathy Accompanied by Behçet Disease.

Purpose: To report a case of acute macular neuroretinopathy (AMN) in Behçet Disease.Case: A 23-year-old male presented with a complaint for central scotoma in his right eye. He had been diagnosed with Behçet Disease 3 years ago. Best-corrected visual acuity (BCVA) was 20/20. Anterior chamber and fundus examinations were unremarkable. Optical coherence tomography revealed a paracentral area of outer nuclear layer thinning. Infrared reflectance showed a well-defined, circular, hyporeflective area. Optical coherence tomography angiography revealed an area of capillary dropout in deep retinal capillary plexus corresponding to that hyporeflective lesion. Microperimetry test showed decreased macular sensitivity on the lesion area and the loss of the macular integrity. In multifocal electroretinogram, diminished amplitudes of the central cone responses were detected nasal to fixation.Conclusion: Behçet disease is a cause of occlusive retinal vasculitis. Accompanied retinal microvascular disease may be a possible risk factor of AMN suggesting ischemic etiopathogenesis for AMN.

Analysis of Parafoveal Microvascular Abnormalities in Behcet's Uveitis Using Projection-Resolved Optical Coherence Tomographic Angiography.

Purpose: To assess the characteristics of parafoveal microvascular abnormalities in Behcet's uveitis (BDU) using projection-resolved optical coherence tomographic angiography (PR-OCTA).Methods: A retrospective study of BDU patients who underwent PR-OCTA examination between April 1, 2017 and October 31, 2018.Results: Sixty consecutive BDU patients (102 eyes) were included. Sixty-two (124 eyes) healthy subjects served as normal controls (NCs). As compared with NCs, the vessel densities (VDs) of superficial and deep retinal capillary plexuses were decreased, and the fovea avascular zone area, perimeter and acircularity index were increased in BDU eyes (all p < .001). Macular edema (p < .001) and long uveitis course (p = .033) were identified as risk factors for capillary disruptions. Consistent VD reductions were observed in the quiescent fellow eyes of BDU patients during unilateral uveitis relapse.Conclusions: BDU patients had significant OCTA changes. Macular circulatory disturbances may exist in the quiescent fellow eyes during unilateral BDU attack.