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.

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.

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.

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.

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.

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.

The clinical features, image findings and risk factors of vena cava syndrome in Behçet's syndrome.

OBJECTIVES: To investigate the clinical features, image findings, and potential risk factors of vena cava syndrome (VCS) in Behçet's syndrome (BS). METHODS: We conducted a case-control study in our BS registry database from 2012 to 2021. Fifty-five BS patients with VCS were enrolled in the case group, and two BS patients without VCS were selected as controls for each VCS case using risk-set-sampling. Multivariable logistic regression was used to detect the risk factors of VCS, and the outcome of these patients was also analysed. We also conducted an exploratory study to evaluate spectral computed tomography (CT) imaging in detecting thrombosis in BS patients with inferior VCS (IVCS). RESULTS: Multivariable logistic regression analysis revealed male gender (OR 11.16, 95%CI 3.34-37.32), early-onset BS (<18 years) (OR 5.57, 95%CI 1.58-19.69), ESR >60 mm/hr (OR 3.83, 95%CI 1.02-12.23) and pathergy reaction (OR 5.10, 95%CI 2.11-12.32) as potential risk factors of VCS in BS patients. For 4 BS patients with IVCS due to thrombosis, spectral CT found better contrast between IVC and thrombi at a low energy level of 40keV using virtual monoenergetic imaging than conventional images at 120kV. With a median follow-up of 3.3 years, the respective estimated 1- and 5-year survival rates were 96.3% and 94.2%, and respective estimated 1- and 5-year relapse-free rates were 93.9% and 78.0%. CONCLUSIONS: Male, early-onset BS, ESR >60 mm/hr, and pathergy reaction are potential risk factors of VCS in BS patients. Spectral CT is valuable in detecting thrombus in vena cava.

Femoral vein wall thickness measurement: A new diagnostic tool for Behçet's disease.

OBJECTIVES: Diagnosing Behçet's disease (BD) is a challenge, especially in countries with a low prevalence. Recently, venous wall thickness (VWT) in lower extremities has been shown to be increased in BD patients. In this study, we aimed to investigate the diagnostic performance of common femoral vein (CFV) thickness measurement in BD and whether it can be used as a diagnostic tool. METHODS: . Patients with BD (n = 152), ankylosing spondylitis (n = 27), systemic vasculitides (n = 23), venous insufficiency (n = 29), antiphospholipid syndrome (APS; n = 43), deep vein thrombosis due to non-inflammatory causes (n = 25) and healthy controls (n = 51) were included in the study. Bilateral CFV thickness was measured with ultrasonography by a radiologist blinded to cases. RESULTS: Bilateral CFV thickness was significantly increased in BD compared with all control groups (P < 0.001 for all). The area under the receiver operating characteristic curve for bilateral CFV thicknesses in all comparator groups was >0.95 for the cut-off value (0.5 mm). This cut-off value also performed well against all control groups with sensitivity rates >90%. The specificity rate was also >80% in all comparator groups except APS (positive predictive value: 79.2-76.5%, negative predictive value: 92-91.8% for right and left CFV, respectively). CONCLUSION: Increased CFV thickness is a distinctive feature of BD and is rarely present in healthy and diseased controls, except APS. Our results suggest that CFV thickness measurement with ultrasonography, a non-invasive radiological modality, can be a diagnostic tool for BD with sensitivity and the specificity rates higher than 80% for the cut-off value ≥0.5 mm.

Deep-learning system for real-time differentiation between Crohn's disease, intestinal Behçet's disease, and intestinal tuberculosis.

BACKGROUND AND AIM: Pattern analysis of big data can provide a superior direction for the clinical differentiation of diseases with similar endoscopic findings. This study aimed to develop a deep-learning algorithm that performs differential diagnosis between intestinal Behçet's disease (BD), Crohn's disease (CD), and intestinal tuberculosis (ITB) using colonoscopy images. METHODS: The typical pattern for each disease was defined as a typical image. We implemented a convolutional neural network (CNN) using Pytorch and visualized a deep-learning model through Gradient-weighted Class Activation Mapping. The performance of the algorithm was evaluated using the area under the receiver operating characteristic curve (AUROC). RESULTS: A total of 6617 colonoscopy images of 211 CD, 299 intestinal BD, and 217 ITB patients were used. The accuracy of the algorithm for discriminating the three diseases (all-images: 65.15% vs typical images: 72.01%, P = 0.024) and discriminating between intestinal BD and CD (all-images: 78.15% vs typical images: 85.62%, P = 0.010) was significantly different between all-images and typical images. The CNN clearly differentiated colonoscopy images of the diseases (AUROC from 0.7846 to 0.8586). Algorithmic prediction AUROC for typical images ranged from 0.8211 to 0.9360. CONCLUSION: This study found that a deep-learning model can discriminate between colonoscopy images of intestinal BD, CD, and ITB. In particular, the algorithm demonstrated superior discrimination ability for typical images. This approach presents a beneficial method for the differential diagnosis of the diseases.

Vascular Behcet's Disease Preceded by Fever of Unknown Origin: Usefulness of Ultrasonography for the Detection of Large-Vessel Vasculitis.

Behcet's disease is a systemic vasculitis characterized by oral and genital ulcers, erythema nodosum, and ocular involvement. Fever of unknown origin is a relatively rare event in Behcet's disease. We present the case of a 17-year-old male patient who suffered from prolonged fever for two months. The patient tested positive for HLA-B52 and levels of acute phase reactants were elevated. He complained of sore throat and neck pain that were evaluated by cervical ultrasonography, which revealed thickening of the carotid arterial wall and narrowing of the vessel lumen. The patient was diagnosed with vascular Behcet's disease and treated with glucocorticoid, which improved the clinical symptoms and thickening of the carotid arterial wall as detected by color duplex ultrasonography. Since vascular Behcet's disease may lead to morbidity and mortality, we suggest the early use of ultrasonography to help detect medium/large-vessel vasculitis. Prolonged fever in patients with Behcet's disease should be promptly evaluated for vascular involvement.

Fragmented QRS complexes are associated with subclinical left ventricular dysfunction in patients with Behcet's disease: Four-dimensional speckle tracking echocardiography.

BACKGROUND: Behcet's disease (BD) is a chronic systemic inflammatory disease in which early detection of cardiac involvement is essential. The aim of this study was to assess the left ventricular (LV) functions in BD patients using four-dimensional (4D) speckle tracking echocardiography (STE) and to test the correlation between LV dysfunction and the presence of QRS fragmentation. METHODS: This cross-sectional study included 64 Behcet's patients and 48 healthy volunteers. The BD group was divided into two subgroups depending on the presence (fQRS+) or absence (fQRS-) of fragmented QRS (fQRS). In both groups, left ventricular global area strain (LV-GAS), global radial strain (GRS), global longitudinal strain (GLS), and global circumferential strain (GCS) were obtained with 4D echocardiography. RESULTS: GAS, GRS, GLS, and GCS values were significantly different in Behcet's patients and in healthy volunteers. GLS and GAS values were lower in the fQRS+ than in the fQRS- group (-15.8 ± 1.8 and -17.9 ± 1.6, P = .001 vs -25.0 ± 3.1 and -29.2 ± 4.2, P < .001, respectively). The duration of disease was longer in fQRS+ than in fQRS- patients (120.8 ± 67.4 vs 71.0 ± 40.5, P < .001). Multiple linear regression analysis showed that fQRS and disease duration were independent predictors of LV-GAS. CONCLUSIONS: Four-dimensional STE may be helpful for the prediction of early cardiac dysfunction in patients with BD. The presence of fQRS may be an indicator of subclinical LV dysfunction.

How to treat myelodysplastic syndrome with clinical features resembling Behçet syndrome: a case-based systematic review.

The association between myelodysplastic syndrome (MDS) and Behçet syndrome (BS) is recognized for over 25 years. High frequency of trisomy 8 and intestinal ulcers are striking features of this association. There are no recommendations for how these patients should be treated. A systematic literature review was performed in PubMed using the keyword combination "(((((intestinal) OR gastrointestinal) OR ulcer) OR Behcet*)) AND ((myelodysplastic syndrome) OR MDS)" in March 2019. Our aim was to gain insight regarding clinical responses to individual treatment modalities. A recent case was also presented and included in the analysis. Data from 41 articles reporting on a total of 53 patients carried adequate information to assess treatment responses. Glucocorticoids provided benefit in 23 of 43 patients. Azacitidine, decitabine, thalidomide, and cyclosporine contributed to a clinical improvement in 4/6, 2/3, 3/4, and 5/8 patients respectively. Hematopoietic stem cell transplantation was successful in 9 of 13 patients. With the use of TNF inhibitors, azathioprine, and mesalamine derivatives, clinical improvement was observed in 3/11, 0/4, and 6/18 patients respectively. Patients with MDS and BS-like features who are resistant to glucocorticoids have so far benefited more from treatment approaches directed at MDS, rather than the immunosuppressive agents used for BS.

Surgical Experience of Behcet's Disease Involving the Peripheral Artery.

BACKGROUND: Behcet's disease with arterial involvement shows rapid poor progression compared with Behcet's disease without arterial involvement, such that it may be thought of as a different disease. With arterial involvement, high rates of postoperative recurrence and anastomotic complications are observed. METHODS: The medical records of 1,361 patients diagnosed with Behcet's disease who visited our hospital from January 2007 to December 2019 were reviewed. Overall, 118 patients with blood vessel involvement were considered. Those with only vein involvement, intracerebral arterial involvement, and coronary arterial involvement were excluded. The 8 patients who underwent surgical treatments and the 9 patients who underwent conservative treatments were included and reported in this study. RESULTS: The mean age and mean follow-up duration of the 17 patients with peripheral arterial involvement were 49.82 ± 13.24 years and 89.85 ± 52.70 months, respectively. In all cases where the operations were performed, the first operation was an emergency operation for rupture of the aneurysm, impending sign of rupturing, or acute ischemia. Eight patients received a total of 18 operations. The average number of operations per patient and the reoperation rate were 2.25 ± 0.89 times and 75%, respectively. The 5-year and 10-year survival rates of the patients who underwent surgery were 75.0% and 30.0%, respectively. When arterial involvement occurs in the form of an aneurysm, the hazard ratio for death is 9.644 (P = 0.040, confidence interval 1.11-83.74). CONCLUSIONS: When the artery invades in the form of an aneurysm, the mortality rate is higher regardless of surgery. The main cause of mortality is complications that occur in the anastomosis sites after the operation. Postoperative short-term and regular imaging of the anastomosis area can be beneficial to detect such complications early to decrease the frequency of emergency surgeries, which will help reduce mortality.

A case with Behçet's disease involving erosive Metacarpophalangeal joint arthritis: the value of ultrasonography in the diagnosis of an Erosion.

BACKGROUND: Behçet's disease (BD) is a chronic multisystemic vasculitis that may emerge with musculoskeletal system involvements, oral-genital recurrent aphthae, mucocutaneous lesions, and ocular symptoms. Arthritis in BD is usually non-erosive and not related to crippling disease. Erosive arthropathy is a very rare manifestation of BD. CASE PRESENTATION: Herein, we present a 60-year-old male patient suffering from BD for 33 years with erosive arthritis in his second metacarpophalangeal joint. After we assessed his finger by X-ray, we diagnosed erosive arthropathy quickly by musculoskeletal ultrasonography. In addition, a systematic literature search was performed via the PubMed and Scopus databases using the keywords, 'Behçet's disease [AND] erosive/destructive arthritis'. CONCLUSIONS: Erosive arthritis due to BD can be evaluated by ultrasonography in an easy, fast and cost-effective manner. The literature search between 1985 and December 2019 revealed a total of 19 patients with peripheral erosive arthropathy related to BD and the characteristics of the results are summarized in the paper.

Ultrasonographic assessment of femoral cartilage thickness in patients with and without uveitis in Behçet's disease.

The objective for this study is to evaluate the femoral cartilage thickness in patients with/without uveitis in Behçet's disease (BD). Patients with BD aged 18-70 years were included. The demographic and clinical characteristics of the patients were recorded. The thickness of femoral articular cartilage was measured with musculoskeletal ultrasound. Cartilage thickness was measured bilaterally from the central points of medial condyle (MFC), lateral condyle (LFC), and intercondylar area (ICA). 20 patients with uveitis [uveitis (+)] and 20 patients without uveitis [uveitis (-)] were included. Both right and left MFC and LFC had statistically significant thinner cartilage in uveitis (+) group (p < 0.05). For the ICA, uveitis (+) patients had thinner femoral cartilage than uveitis (-) patients; however, in the left side, statistically significance could not be detected. Femoral cartilage was thinner in uveitis (+) patients than in uveitis (-) patients in BD. This relationship between uveitis and femoral cartilage thickness may be helpful in the prevention and early treatment of cartilage degeneration.

Behcet's disease presenting with pulmonary thromboembolism.

Behcet's disease is a chronic inflammatory multi-systemic vasculitis. Recurrent oral and genital aphthous ulcers, uveitis, arthritis, arterial aneurysms, venous thrombosis, gastrointestinal system lesions and skin lesions can be seen. Large, medium or small arteries and veins may be involved. While venous lesions cause occlusion, arterial lesions can cause both occlusion and aneurysm. Major forms of pulmonary involvement include pulmonary artery aneurysm, arterial and venous thrombosis, pulmonary infarction, recurrent pneumonia, and pleurisy. In Behcet's disease, pulmonary thromboembolism (PE) is often associated with endothelial damage. Neutrophils play an important role in the pathogenesis of thrombosis. Coagulation components such as fibrinogen, thrombin, factor Xa and factor VIIa activate the inflammatory cascade and induce vascular events. It is important to understand the pathogenesis of vascular events in determining the effective treatment strategy. Here we present a patient with Behcet's disease who was presented with pulmonary embolism and was investigated for the etiology of thrombosis.

Nervous system involvement in Behçet's syndrome.

PURPOSE OF REVIEW: Neurological involvement in Behçet's syndrome is defined as 'the occurrence of neurological symptoms and signs in a patient who meets the International Diagnostic Criteria for BS not otherwise explained by any other known systemic or neurological disease or treatment, and in whom objective abnormalities consistent with neuro-Behçet's syndrome (NBS) are detected either on neurological examination, neuroimaging studies (magnetic resonance imaging [MRI]), and/or on cerebrospinal fluid (CSF) examination'. Given that the neurological involvement of Behçet's syndrome carries a poor prognosis, we aimed to describe the differential diagnosis of NBS and highlight the different radiological patterns together with the treatment options. RECENT FINDINGS: Two distinct MRI patterns of spinal cord involvement in Behçet's syndrome according to T2-weighted axial images were described: 'Bagel Sign' pattern: a central lesion with hypointense core and hyperintense rim with or without contrast enhancement; and 'Motor Neuron' pattern: a symmetric involvement of the anterior horn cells. Infliximab prevents patients from having further attacks and even led to improvement in the neurological examination. SUMMARY: As the treatment options completely differ, a NBS diagnosis should be carefully made in patients with clinical and MRI features mimicking other central nervous system inflammatory disorders.

What is the role of diffusion tensor imaging (DTI) in detecting subclinical pyramidal tract dysfunction in Behçet's and neuro-Behçet's cases?

The aim of this study is to investigate the pyramidal tract integrity with DTI in Behçet's and neuro-Behçet's cases. We performed this technique in two subgroups of neuro-Behçet's patients (parenchymal and vascular), and Behçet's cases without neurological involvement and control group. Totally, 28 patients were investigated. The control group was composed of 14 healthy people. Cranial MR and DTI were performed in three patient groups and the control group. At DTI, circular regions of interest (ROI) were symmetrically drawn on axial slices on the left and right sides along the pyramidal tract pathway at two levels: middle one third of the cerebral peduncle and posterior limb of the internal capsule. Fractional anisotropy (FA) values for each ROI were obtained by averaging all voxels within the ROI. Calculated FA values on both sides (left and right) of the posterior limb of the internal capsule and cerebral peduncle are significantly lower in all three patient groups when compared to the control group. But there is no any difference of FA values in the selected brain regions of three patient groups. FA values on the posterior limb of the internal capsule and cerebral peduncle do not show a statistically significant difference in parenchymal neuro-Behçet's cases. Our study demonstrates that DTI can detect subclinical pyramidal tract dysfunction in neuro-Behçet's and Behçet's patients. Detection of subclinical nervous system involvement is crucial for morbidity in Behçet's disease. For this reason, studies based on DTI, which include a large number of patients and explore different brain regions, are needed to guide clinicians.