The clinical and radiological features and prevalence of Neuro-Behçet's Disease: A retrospective cohort multicenter study in Saudi Arabia.

BACKGROUND: Neuro-Behçet's disease (NBD) is defined as primary neurological involvement in patients with systemic symptoms of BD. The variety of clinical presentations seen in NBD and the long list of similar conditions make diagnosis challenging. This retrospective study aimed to estimate the prevalence and describe neurological involvement in patients with Behçet's disease who presented to King Abdulaziz Medical Cities in Jeddah and Riyadh, Saudi Arabia. METHODS: This was a retrospective, cohort study which utilized a non-probability consecutive sampling technique to include all patients diagnosed with NBD patients. All patients with BD (215) were screened for neurological symptoms. Thirty-five patients were found to be diagnosed with NBD. Outcomes were estimated using the modified Rankin scale (mRS). RESULTS: In our cohort, one in six patients with BD was diagnosed with NBD. A total of 35 patients were diagnosed with NBD (mean age 27.56 ± 10.36 years; [2.88:1; Male: Female]). The main clinical features of NBD were headaches, weakness, unsteadiness, and dysarthria. The most commonly involved sites on imaging were the brainstem, diencephalon, cerebellum and basal ganglia. Oligocolonal bands were negative in all patients. Maintenance therapy most commonly included oral corticosteroids, azathioprine, and/or infliximab. Most patients received pulse corticosteroids alone when presenting with acute relapse. Half of our cohort was asymptomatic and three in four had favorable outcomes. CONCLUSION: NBD is common among patients with BD in our population with most patients having favorable outcomes. Patients might have a wide array of symptoms which might make the diagnosis challenging.

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.

Increased inferior vena cava wall thickness as a sign of extensive venous inflammation in Behçet's Disease.

INTRODUCTION/OBJECTIVES: Behçet's disease (BD) affects both arterial and venous vessels. We have previously shown that common femoral vein wall thickness (WT) is increased in BD and can be used as a diagnostic test. However, there is limited data assessing large veins. Therefore, this study seeks to assess inferior vena cava wall thickness (IVC) by transthoracic echocardiography (TTE) in BD compared to healthy controls (HC). METHODS: Age- and gender-matched 70 BD patients and 51 HC were included. IVC wall thickness and common femoral vein WT were measured by TTE and Doppler ultrasonography, respectively. All examinations were performed on the same day as the clinical assessment. RESULTS: The mean IVC wall thickness of BD patients was significantly higher than HC (2.9 mm (0.3) vs 2.6 mm (0.3), p < 0.001). Patients with mucocutaneous involvement (2.8 mm (0.3)) and major organ involvement (2.9 mm (0.3)) had significantly thicker walls compared to HC (p = 0.003, p < 0.001, respectively). IVC wall thickness was higher in patients with vascular involvement compared to those with nonvascular major organ involvement (3.1 mm (0.3) vs 2.8 mm (0.2), p = 0.04). There was a moderate correlation between IVC and common femoral vein WT (r = 0.49 for the right, r = 0.43 for the left, p = 0.01 for both). CONCLUSION: This study shows that venous wall inflammation is not limited to lower extremity veins and is also present in IVC walls of BD patients regardless of IVC involvement. Vascular wall inflammation is probably a widespread vascular event of all venous walls in BD. Key Points • Venous wall inflammation is not limited to lower extremity veins and is present also in IVC wall in Behçet's disease. • Extensive venous wall inflammation in Behçet's disease includes large venous structures despite not being clinically involved.

Characteristics of Cerebral Venous Sinus Thrombosis Due to Autoimmune Diseases: A Single-Center Retrospective Observational Study.

OBJECTIVES: Cerebral venous sinus thrombosis (CVST) is a cerebrovascular disease characterized by thrombosis of the cerebral venous or dural sinuses. Autoimmune diseases (AD) are important causes of CVST. This study aims to reveal the differences between CVST associated with autoimmune diseases compared with other causes (OCs) and Behcet's syndrome (BS) compared with other ADs. METHODS: This is a single-center retrospective study in which the medical records of 187 patients we followed with a diagnosis of CVST between 2008 and 2023 were collected retrospectively. Four neurologists collected data on initial symptoms, neurological examinations, and laboratory findings. Findings on magnetic resonance imaging and magnetic resonance venography performed on all patients (thrombosis localizations, hemorrhagic or ischemic complications, and collateralization) were re-evaluated by 2 radiologists. The results were compared with AD, other ADs, and OCs groups. RESULTS: There were 28 cases of CVST associated with AD. Of these, 18 were BS, and 10 were other AD. Subacute-chronic onset, headache, and transverse sinus involvement were more common in AD-related patients than in OCs. However, collateralization, venous infarction, hemorrhagic transformation, and bleeding were less common. BS-related patients had earlier age, more frequent transverse sinus, less frequent cortical vein thrombosis, and better collateralization than other ADs. CONCLUSION: CVST is one of the rare complications in autoimmune diseases. It has a more subacute-chronic onset. Since headaches are more common, it is essential to make a differential diagnosis of CVST in autoimmune diseases with chronic headaches. Transverse sinus thrombosis is more common. Collateralization, venous infarction, and hemorrhagic transformation are less.

Comparative analysis of optical coherence tomography angiography (OCTA) results between Behçet's disease patients and a healthy control group.

PURPOSE: This study aimed to compare optical coherence tomography angiography (OCTA) findings between patients with Behçet's disease (BD) and individuals with healthy eyes. DESIGN: A cross-sectional study. METHODS: This cross-sectional study was conducted on patients (67 eyes) with BD who were referred to Feiz Hospital and healthy eyes (43 eyes). All subjects underwent Snellen visual acuity, a slit-lamp examination, measuring intraocular pressure, conducting a dilated fundus examination, OCTA imaging, and spectral-domain (SD)-OCT imaging. OCTA retinal vascular measurements including optic nerve VD, macular-associated VD( superficial and deep), foveal avascular zone (FAZ) area, FAZ perimeter (PERIM), and vessel density within a 300-µm-wide region of the FAZ (FD) were compared between the groups. RESULTS: A significant difference was evident between the two groups (healthy one group and BD group) in terms of parafoveal and perifoveal total retinal thickness, total pRNFL VD in all quadrants except the inferior sector (P < 0.05), and macular superficial, and deep VD in all regions except temporal and superior perifoveal VD (P < 0.05) following adjustments for age, gender, and signal strength index. When comparing the two groups, ocular Behçet's disease (BD) and non-ocular BD, it was evident that peripapillary vessel density (VD) exhibited a significant decrease in ocular BD eyes in all sectors except for the superior and inferior ones, as compared to non-ocular BD eyes. In addition, the comparison of ocular BD and non-ocular BD showed superficial and deep VDs were lower in ocular BD than non-ocular BD in all regions. CONCLUSION: According to these findings, peripapillary and macular vessel density is affected in BD. Key Points • The study utilized OCTA to compare retinal features in Behçet's disease (BD) patients and healthy individuals, revealing significant differences in retinal thickness and vessel density. • Ocular BD demonstrated reduced peripapillary vessel density compared to non-ocular BD. • The demonstrated association between ADMA and cIMT in patients with early SSc may suggest a role of NO/ADMA pathway in the initiation of macrovascular injury in SSc.

Imaging characteristics and prognostic factors of Behcet's disease with arterial involvement: A long-term follow-up study.

PURPOSE: To investigate the imaging characteristics and prognostic factors for the long-term survival of Behcet's disease (BD) with arterial involvement. METHODS: In this retrospective study, BD patients with arterial involvement were identified from January 2003 to January 2020. Arterial lesions were detected by ultrasonography, traditional arteriography, and/or computed tomography angiography (CTA). Cox proportional hazards regression analyses were performed to identify the prognostic factors. RESULTS: Totally, 84 BD patients with arterial involvement were identified (73.8 % males). The mean age at BD diagnosis was 39.1 ± 13.1 years. Arterial involvement was the initial manifestation in 33.3 % of the patients, and the median time from BD diagnosis to arterial involvement was 6 (IQR 1-15.5) years for the rest of patients. Systemic artery involvement and pulmonary artery involvement (PAI) were found in 64 and 27 patients, respectively. Approximately 94.0 % (79/84) of the patients had more than one artery involved concurrently or successively during the course of BD. Aneurysm/dilation was the most prevalent lesion in the aorta (76.0 %), while stenosis/occlusion was the main lesion of the coronary artery (90.9 %) and other aortic branches (74.5 %). Pulmonary hypertension was found in 70.4 % (19/27) of patients with PAI. The 5- and 10-year survival rates of BD patients with arterial involvement were 87.4 % and 84.1 %, respectively. Cardiac involvement (HR: 4.34) and pulmonary artery aneurysm/dilation (HR: 4.89) were independently associated with mortality. CONCLUSIONS: Arterial lesions associated with BD usually involve multiple arteries and manifest differently in different types of arteries. Cardiac involvement and pulmonary artery aneurysm/dilation are independent prognostic factors of BD patients with arterial involvement.

Life-Threatening Involvement of Bilateral Pulmonary Arteries in Behçet Disease on FDG PET.

ABSTRACT: A 29-year-old man with Behçet disease exhibiting lower-limb swelling and dyspnea underwent 18 F-FDG PET/CT. The imaging revealed bilateral pulmonary artery involvement, and pulmonary artery aneurysms and mural thrombus were confirmed by CT pulmonary angiography. This case underscores the utility of 18 F-FDG PET in identifying life-threatening vasculitis complications in Behçet disease.

Pulmonary hypertension in Behçet's disease: echocardiographic screening and multidisciplinary approach.

BACKGROUND: Little is known about the prevalence and causes of pulmonary hypertension (PH) in Behçet's disease (BD). This study was conducted to determine the prevalence and causes of PH in BD. METHODS: In this descriptive study, we screened 154 patients with BD for PH using transthoracic echocardiography between February 2017 and October 2017. An estimated systolic pulmonary arterial pressure (sPAP ≥ 40 mmHg) was used as the cutoff value to define PH. Patients with BD were categorized into 5 groups according to organ involvement including mucocutaneous/ articular, ocular, vascular, gastrointestinal, and neurologic involvement. Additional laboratory and imaging results were obtained from hospital file records to determine the causes of PH. RESULTS: PH was detected in 17 (11%) patients. Nine (52.9%) of these patients had group II PH (due to left heart disease), 4 (23.5%) had IV PH (due to pulmonary arterial involvement), and 1 had III PH (due to chronic obstructive lung disease). The frequency of PH was higher in BD patients with vascular involvement than those without (52.9% vs 28.5%; p = 0.04). Among 10 patients with pulmonary artery involvement (PAI) 4 (40%) had PH. Although the vascular BD group had the highest rate of PH, we observed no statistically significant difference in the frequency of PH between the predefined BD subgroups. DISCUSSION: : PH is not rare in patients with BD. The majority of BD patients with PH are in group II or IV PH. Patients with vascularinvolvement carry a higher risk for the development of PH. Monitoring BD patients with PAI should be considered for the development of group IV PH.

Intima-media thickness of common femoral vein is increased in Behçet's disease.

The histopathological data about vascular inflammation in Behçet's disease(BD) mainly comprises patients with arterial involvement. Inflammatory cell infiltration was mainly observed around the vasa vasorum and adventitial layer of the aneurysmatic vessels, and only a few cells were seen in the intimal layer during active arteritis. There is limited data for the histopathology of venous inflammation. We recently showed that increased common femoral vein(CFV) wall thickness is a specific sign of vein wall inflammation in BD. We aimed to investigate the different vein subsections measuring the whole wall and the intima-media thickness(IMT) of CFV with ultrasonography in BD. We found increased IMT of CFV compared to controls as well as CFV wall thickness. This study shows that there is a full layer venous wall inflammation in BD independent of vascular involvement. Our results suggest that venous endothelial inflammation may trigger the thickening of the vein wall and cause thrombotic tendency in BD.

Bagel sign in neuro Behçet's disease myelitis: neuroimages.

Neuro-Behçet's disease (NBD) is a chronic heterogenous autoimmune disorder. It may involve central or pripheral nervous system but rarely shows spinal cord involvement (SCI). Bagel Sign is a unique sign of SCI due to NBD. It is a central T2W hyperintense lesion with a hypointense core on axial magnetic resonance imaging (MRI). This sign may be a complete or an incomplete ring with or without post-contrast enhancement. Here we report a patient with NBD whose primary presentaion was transverse myelitis. A 14-year-old patient was admitted due to triparesis and urinary retention. He had T2W hyperintensities from the left basal ganglion down to the T10 level. A similar anteromedial spot has been described in anterior spinal cord infarction which favors an ischemic pathogenesis for NBD. To our knowledge this is the first report of Bagel Sign and longitudinally extensive transverse myelitis with gray matter involvement.

Vascular involvement in Behçet's disease: ultrasound assessment of femoral vein intima-media thickness, nailfold capillaroscopy and endothelial progenitor cells in a national referral centre.

OBJECTIVES: To assess vascular involvement at different levels in patients with Behçet's disease (BD). METHODS: We conducted an observational study of unselected consecutive patients with BD assessed in a national referral centre. Demographic and clinical variables were collected. Common femoral vein (CFV) intima-media thickness (IMT) was measured with a high-resolution Doppler ultrasound (US). Microvascular involvement was evaluated performing a nailfold capillaroscopy (NFC). Endothelial progenitor cells (EPC) were measured in peripheral blood of patients and healthy controls (HC) by flow cytometry. RESULTS: A total of 42 patients with BD were evaluated. Clinical vascular manifestations were present in 17 of them. Arterial hypertension was associated with a higher frequency of vascular manifestations (p=0.003). The median [IQR] value of the CFV IMT was significantly higher in patients with vascular manifestations (0.65 [0.45-0.82] vs 0.49 [0.39-0.55]; p= 0.028). The NFC examination was abnormal in 54.8% of the patients, being the most common findings: capillary loop dilation (45.2%), mega capillaries (21.4%) and micro haemorrhages (16.7%). A significant increase in EPC frequency was observed in patients with BD when compared with HC (p=0.011). CONCLUSIONS: The assessment of CFV IMT with Doppler US constitutes a useful technique to evaluate clinical vascular involvement in BD patients. Microvascular involvement is not uncommon in BD and can be easily assessed by NFC. Furthermore, EPC may be a useful blood biomarker of the disease.

Uveitis in Behçet disease - an update.

PURPOSE OF REVIEW: Uveitis is a major manifestation of Behçet disease (BD) and potentially has a high morbidity. This article reviews recently published data on BD uveitis. RECENT FINDINGS: A set of classification criteria and a diagnostic algorithm have been developed for BD uveitis. Recent reports have confirmed male predominance and posterior segment inflammation in the majority of BD uveitis patients. A high uveitis attack severity score, fluorescein angiographic leakage at the posterior pole, and disruption of outer retinal layers on optical coherence tomography (OCT) predict poor visual outcome. OCT-angiography studies have suggested subclinical changes of retinal capillaries in patients with or without ocular involvement. In a randomized controlled trial, interferon-α was superior to cyclosporine. Favorable outcomes were reported with earlier initiation, optimization, and withdrawal of infliximab after remission. Adalimumab as first-line was superior to conventional therapy. SUMMARY: Classification criteria will be used to select a homogeneous group of patients for research and the diagnostic algorithm may help ophthalmologists predict the probability of BD uveitis based on ocular findings. Fluorescein angiography and OCT are the routine imaging modalities. Clinical relevance of OCT-angiography is unclear. Interferon-α, infliximab, and adalimumab have proven superior efficacy compared to conventional therapy.

Transthoracic echocardiographic assessment of cardiac valves in patients with Behçet's disease.

The main objectives of the current study are to investigate valvular involvement in patients with cardiac Behçet's disease (BD) and find out the risk factors of valvular involvement in cardiac BD. We retrospectively assessed the clinical and echocardiographic data in the medical records of 121 patients with BD admitted to Beijing Anzhen Hospital from January 2015 to January 2022. We evaluated the valvular structure and function mainly by echocardiography. A total of 77 BD patients (77/121, 63.64%) had cardiac valvular involvement. Valvular lesions occurred more frequently in males (p = 0.022). Aortic regurgitation (AR) (62/77, 80.52%) was the most common finding and severe AR occupied 80.65% (50/62). The most common manifestations of BD patients with severe AR was aortic valve prolapse (25/50, 50%), followed by echo-free spaces within the aortic annulus (11/50, 22%), vegetation-like lesions (10/50, 20%), and aortic root aneurysm (10/50, 20%). The incidence of paravalvular leaks (PVL) in BD patients was 14.29% (7/49). The diameter of the sinus of Valsalva and proximal ascending aorta, and total cholesterol (TCHO) were the independent risk factors of moderate-severe aortic valvular regurgitation (p < 0.01). Left ventricular end-diastolic dimension (LVEDD), left ventricular ejection fraction (LVEF) and brain natriuretic peptide (BNP) were significantly associated with moderate-severe mitral valvular regurgitation (p < 0.01). The most common valvular abnormality in BD is AR. Echocardiography has great value in the comprehensive evaluation and accurate diagnosis of valvular involvement in BD patients.

Claude Syndrome in Childhood Associated with Probable Neuro-Behcet Disease.

Claude syndrome is a rare midbrain stroke syndrome characterized by ipsilateral third cranial nerve palsy and contralateral hemiataxia. So far, only a few cases have been reported in childhood. We present two children with Claude syndrome at 9 and 15 years of age. The typical clinical picture was consistent with brain magnetic resonance imaging findings. A thorough investigation regarding the underlying etiology revealed no definite diagnosis but clues suggestive of probable neuro-Behcet disease. Awareness of pediatric neurologists on arterial ischemic stroke has been increasing over the past decades, enabling timely diagnosis and appropriate management of rare childhood cases with midbrain stroke.

Giant Vertebral Artery Aneurysm in Behçet's Disease: A Rare Case and Review of the Literature.

BACKGROUND: Behçet's disease is a chronic inflammatory disease that involves systemic organs and vascular structures. Arterial system involvement is rarer than the venous system, and it is one of the major causes of death. Vertebral artery (VA) involvement is rare and there are a limited number of reported cases in the literature. This report aimed to present the imaging findings of a case of Behçet's disease with a giant aneurysm originating from the right VA. CASE REPORT: A 35-year-old woman with a 10-year history of Behçet's disease applied to our center with complaints of pain on the right side of the neck. CT angiography showed a 7.5x6.5 cm sized saccular aneurysm in the superior of the right hemithorax adjacent to the mediastinum, with contrast filling the V1 segment of the VA. In our case, the VA aneurysm had no relation to the subclavian artery and showed filling from the contralateral VA. The described findings were demonstrated in Doppler examination, phase-contrast MRI, and conventional and CT angiography. CONCLUSION: Behçet's disease is a chronic inflammatory disease that can involve the arterial system. Although VA involvement has not been reported frequently, this vasculitic process may affect all arterial structures.

Echocardiography Parameters in Behcet's Disease, A Comparative Study.

OBJECTIVE: Behçet's disease (BD) is a chronic inflammatory disease with multiple organ involvements. Although cardiac involvement is not common, it can increase patient morbidity and mortality and decrease life quality. In the present study, echocardiographic abnormalities in BD with no cardiac symptoms were investigated. METHODS: This cross-sectional descriptive-analytic study was performed on patients referred to Imam Reza Hospital in Mashhad from 2015 to 2018. The participants were divided into control and BD groups. Patients with BD were diagnosed based on ISG criteria. All participants underwent transthoracic echocardiography. Echocardiographic parameters were compared between BD and control groups. RESULTS: In this study, the severity of aortic (AR), mitral (MR), and tricuspid valve regurgitation (TR) was higher in BD than in the control group, while only TR severity showed a statistically significant difference (p-value < 0.001). Systolic Pulmonary Artery Pressure (sPAP) was significantly higher in BD compared to the control group (24.6 mmHg versus 22.81 mmHg, respectively) (pvalue = 0.019). CONCLUSION: It seems echocardiography is valuable in evaluating a cardiac function, even in asymptomatic patients. Cases with moderate mitral and aortic regurgitation were only observed in BD. Long-term follow-up is suggested, and further studies are required.

Exists a role for serum irisin in Egyptian Behcet's patients with subclinical atherosclerosis?

OBJECTIVES: To examine the serum irisin level in a group of Behcet's disease patients, its association with illness parameters, and its utility in diagnosing subclinical atherosclerosis. METHODS: This randomized case-control study included 50 patients and 50 age- and sex-matched controls. Carotid Doppler ultrasound for the measurement of the carotid artery intima-media thickness (CIMT) and ankle-brachial pressure index (ABPI) were performed. A clinical evaluation, lipogram, and serum irisin were also performed. RESULTS: Between the patients and the control group, there was a significant difference in CIMT, S. irisin level, and ankle-brachial pressure index; however, gender and BMI did not significantly affect CIMT, ABPI, or S. irisin level. CIMT demonstrated a substantial negative correlation with both S. irisin and ABPI (r = - 0.62, P 0.0001). With a sensitivity of up to 94.30% and a specificity of 93.30%, the ROC analysis revealed that a decrease in S. irisin level in Behcet's patients was indicative of subclinical atherosclerosis. The drop in the ABPI level demonstrated a sensitivity of up to 94.30% and a specificity of 100%. CONCLUSION: Subclinical atherosclerosis is prevalent among Egyptian Behcet's patients, and S. irisin can be employed as a biomarker for diagnosing subclinical atherosclerosis in Behcet's illness. Key Points • Serum irisin has been studied in numerous autoimmune disorders as a marker for subclinical atherosclerosis, although its importance in Behcet's disease remains unclear (BD). • We examined the change in serum irisin levels in Behcet's disease patients and healthy controls. In addition, its association with carotid artery intima-media thickness (CIMT) and ankle-brachial pressure index was investigated (ABPI). • Changes in serum irisin levels are significant in BD, and a decrease in irisin level indicates subclinical atherosclerosis.