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.

Utilizing shear wave elastography for the evaluation of optical nerve involvement in Behçet's disease.

In this study, we investigated the elasticity of the optic nerve using shear wave elastography (SWE) in patients with Behçet's Disease (BD) and compare findings with a healthy control group. Optic nerve measurements of 40 eyes in the BD group and 38 eyes in the control group were performed by SWE (SWE-right and SWE-left). The mean age of the individuals in the BD group was higher than controls (p = 0.022). The SWE measurement results in the right eye were significantly higher in the BD group (p = 0.005), while SWE-left values were similar in the two groups (p = 0.206). There was a moderate positive correlation between age and SWE-right (r = 0.347, p = 0.031), there was no correlation between SWE-left and age (p = 0.756). It was found that SWE-right had significant value in distinguishing between BD and healthy controls (sensitivity: 75%, specificity: 78.9%; p = 0.005); whereas, SWE-left did not (p = 0.206). In the multiple logistic regression analysis, after adjusting for age and sex, the performance of SWE-right to distinguish BD patients from controls was found to remain significant (OR: 15.045, 95% CI: 2.33-97.120, p = 0.004). Due to the fact that only SWE-right values appear to have significant discriminatory performance for BD, it is feasible to suggest that the frequency and severity of BD involvement are associated with SWE-related changes in the optic nerve. This finding indicates the need for more comprehensive studies evaluating optic nerve SWE values in this context.

Giant Left Anterior Descending Coronary Artery Aneurysm in a Patient with Behçet's Disease.

Coronary artery involvement is quite rare in the course of Behçet's disease. Complications secondary to coronary artery aneurysms, including rupture, dissection, and myocardial ischemia, may be fatal. In young patients without cardiovascular risk factors, systemic inflammatory vasculitis syndromes should be investigated in case of acute coronary syndrome secondary to dilated coronary arteries. In this report, we present our management strategy in a 31-year-old male patient with Bechet's disease.

Retinal microvasculature features in patients with Behcet's disease: a systematic review and meta-analysis.

This meta-analysis aimed to analyze retinal microvasculature features in eyes with Behçet's disease (BD) using optical coherence tomography angiography (OCTA). Electronic databases, including PubMed, Web of Science, Embase, and Cochrane Library, were comprehensively searched for published studies comparing retinal microvasculature characteristics between eyes with BD and controls. Continuous variables were calculated using the mean difference (MD) with 95% confidence interval (CI). Review Manager software (version 5.30) was used to conduct statistical analysis. A total of 13 eligible studies involving 599 eyes with BD and 622 control eyes were included in the meta-analysis. The pooled results showed that the macular whole enface superficial and deep vessel density (VD) values measured by OCTA were significantly lower in eyes with BD than in control eyes (superficial VD: MD = - 3.05, P < 0.00001; deep VD: MD = - 4.05, P = 0.0004). The foveal superficial and deep VD values were also significantly lower in the BD group than in the control group (superficial VD: MD = - 1.50, P = 0.009; deep VD: MD = - 4.25, - = 0.03). Similarly, the analysis revealed a significant reduction in the parafoveal superficial and deep VD in eyes with BD than in control eyes (superficial VD: MD = - 3.68, P < 0.00001; deep VD: MD = - 4.95, P = 0.0007). In addition, the superficial and deep foveal avascular zones (FAZs) were significantly larger in patients with BD than in controls (superficial FAZ: MD = 0.06, P = 0.02; deep FAZ: MD = 0.12, P = 0.03). The present meta-analysis found that macular whole enface VD, foveal VD, and parafoveal VD were lower in eyes with BD, and the FAZ was larger in patients with BD. The findings suggest that OCTA can assist clinicians in diagnosing and monitoring the status of patients with BD.

Cardiac magnetic resonance imaging in patients with Behçet's disease.

BACKGROUND: Behçet's disease (BD) is a multisystemic vasculitis that may affect the heart. However, the incidence and nature of cardiac involvement in BD have not been clearly documented yet. The aim of this study was to delineate the cardiac magnetic resonance imaging (MRI) appearances of cardiac involvement in BD patients. METHODS: This cross-sectional observational study was carried out 30 BD patients without known cardiac disease. Patients were subjected to history taking, physical examination, echocardiography and cardiac MRI. RESULTS: At least one abnormality on cardiac MRI was observed in 20/30 patients (66.67%). Myocardial oedema was observed in 3 patients (10%) and late gadolinium enhancement in 1 patient (3.3%). Pericardial effusion was found in 3 patients (10.0%), global hypokinesia in 6 patients (20.0%) and intra-cardiac thrombosis in only 1 patient (3.3%). Pulmonary artery was dilated in 4 patients (13.3%). Left ventricular (LV) and right ventricular (RV) end diastolic volume were altered in 4 patients (13.3%) and 7 patients (23.3%) respectively. LV and RV end systolic volume were abnormal in 7 patients (23.3%) and 5 patients (16.7%) respectively. There was aortic valve regurge in 2 patients (6.7%), tricuspid valve regurge in 9 patients (30%), and mitral valve regurge in 9 patients (30%). Dilated left main coronary artery was found in 2 patients (6.7%) and arrhythmogenic right ventricular dysplasia in only one patient 1 patient (3.3%). On logistic regression analysis, BD activity index score was a significant predictor of cardiac abnormalities. CONCLUSION: BD may cause cardiac abnormalities without clinical manifestations and cardiac MRI may represent a tool for early detection of these subtle abnormalities. Higher BD activity index scores are strongly linked to cardiac problems.

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.

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.

Vocal cord involvement and hoarseness in a patient with Behcets disease: A rare case report.

RATIONALE: Behcets disease (BD) is a type of chronic systemic vasculitis that typically manifests as a mucocutaneous disease with orogenital ulcers, skin damage, and uveitis. The clinical diagnosis is often difficult because of the diversity of organs that may be involved and lack of specific pathological diagnosis. PATIENT CONCERNS: A 26-year-old woman presented as a nearly 2-week history of hoarseness with throat pain. DIAGNOSES: In the present case, Fiber laryngoscopy showed multiple ulcers involving the epiglottic tubercle, bilateral false vocal cord, middle area of the left vocal cord, and full length of the right vocal cord. Multidisciplinary physicians combined the patients clinical manifestations and pathological findings to make the Behcets disease diagnosis. INTERVENTIONS: As the diagnosis confirmed, immediately began appropriate medical therapy (prednisolone at 30 mg once per day and thalidomide at 50 mg once per night in a month). OUTCOMES: The ulcer on the right vocal cord disappeared but left a scar. Therefore, the patient experienced only partial recovery from the hoarseness. LESSONS: Behcets disease can cause damage to multiple organs. Although the combination of vocal cord ulcers and hoarseness is rare in patients with BD and has not been previously reported to date, such patients should be treated with caution in clinical practice.

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.

Behcet's disease involved the root of aorta in the treatment with modified Bentall procedure: a case report.

BACKGROUND: Behcet's disease (BD) is a multisystemic vasculitis of unknown etiology, the incidence of cardiovascular system involvement is rare, about1-5% (Sakane et al., N Engl J Med 341:1284-91, 1999). BD combined with aortic pseudoaneurysm and aortic valve regurgitation is usually need surgical treatment, but there is controversy about which surgical method to choose. CASE PRESENTATION: We report a case of BD combined with severe aortic valve regurgitation and two giant pseudoaneurysms of the aortic sinus. The patient underwent modified Bentall procedure (MBP) and use oral immunosuppressive as well as corticosteroid strictly, after 8 months follow-up, the patient recovered well. CONCLUSION: For patient with aortic valve regurgitation and ascend aortic pseudoaneurysm caused by BD, we recommend modified Bentall procedure when rheumatism in a stable period. Corticosteroids and immunosuppressive drugs should be used before and after surgery.

Neuro-Behcet Disease Mimicking Lymphoma of the Brain and Coexisting with A Dural Arteriovenous Fistula: A Case Report.

BACKGROUND: Diverse clinical manifestations have characterized reports of central nervous system involvement in Behcet disease. There is a limited number of published case reports of neuro-Behcet disease mimicking brain tumor. CASE DESCRIPTION: We present a rare case of neuro-Behcet disease exhibiting mixed systemic and neurological symptoms mimicking malignant lymphoma. Delayed presentation of oral aphthae and rapid improvement of symptoms with steroid treatment led to a diagnosis of neuro-Behcet disease without brain biopsy. The patient also underwent surgery for the resection of an arterio-venous fistula. CONCLUSIONS: We recommend management of neuro-Behcet disease based on perceived natural history risks, with the understanding that no data exist on this within patients population. A multidisciplinary team approach is always recommended because neuro-Behcet disease is an uncommon condition, especially in neurosurgical practice.

[Spontaneous aortic isthmus aneurysm: Exceptional revelation of Behçet's disease]. / Anévrisme spontané de l'isthme aortique révélant une maladie de Behçet.

Arterial involvement in Behçet's disease is rare. The exceptional aortic isthmus location is difficult to treat. We report the case of a young man in whom an aortic isthmus aneurysm revealed Behçet's disease. In this exceptional situation, we opted for classical open surgical repair despite current preference for endovascular treatment.

One year in review 2018: Behçet's syndrome.

New epidemiologic studies from Poland, Jordan, Algeria, Taiwan and Korea highlight the geographic differences in incidence, prevalence and clinical features of Behçet's syndrome (BS). A study from Austria comparing clinical manifestations of their BS patients with different countries of origin suggest that environmental factors may be important in the disease phenotype of BS. New genetic association studies dealing with the innate and acquired aspects of BS prevailed during 2017 and novel susceptibility and regulatory factors were described. Common denominators among various disease processes were again highlighted and epigenetic factors were emphasised. "Bagel sign" pattern, a central lesion with hypo-intense core and hyper-intense rim was defined in the spinal MRIs of the patients with neuro-BS especially during the acute attacks of myelopathy. This distinctive pattern suggests venous thrombosis and surrounding oedema in the spinal cord. Pseudotumour cerebri may present with similar clinical presentation to that observed in cerebral venous sinus thrombosis, responds well to immunosuppressive treatment, and could be associated with venous thrombotic relapses. Menstruation and certain food appear to exacerbate skin and mucosa lesions in BS. The EULAR recommendations for the treatment of BS have been updated with 5 new overarching principles and one additional recommendation for surgical management of vascular complications. Infliximab initiated earlier in the course of uveitis yields a better visual outcome. Tapering or stopping of anti-TNF agents seem to be possible when remission has been achieved. Adalimumab appears to be more effective for venous thrombosis than classical immunosuppressives. Oral anticoagulants might not be crucial for cerebral or peripheral venous thrombosis. Transcatheter embolisation of pulmonary aneurysms may be life-saving by providing immediate control of haemoptysis. The results of surgery for pulmonary artery involvement appear to be satisfactory.

Intestinal Behçet Disease: Evaluation With MR Enterography-A Case-Control Study.

OBJECTIVE: The purposes of this study were to discern imaging findings that distinguish Behçet disease from small-bowel Crohn disease, to find initial performance estimates for these findings, and to evaluate the diagnostic value of MR enterography (MRE) for detecting intestinal Behçet disease. MATERIALS AND METHODS: The MRE examinations of 30 consecutively registered patients with established intestinal Behçet disease were reviewed by two blinded readers. The frequencies of MRE findings were compared with those obtained for 30 control subjects with small-bowel Crohn disease who were matched for sex and age. The performance estimates were generated with ileocolonoscopic and histopathologic findings as the reference standard. RESULTS: Polypoid pattern and homogeneous mural enhancement were the findings seen more frequently in Behçet disease (p = 0.000) than in Crohn disease (p = 0.003). Stricture formation, long-segment disease, and involvement of more proximal ileal segments favored small-bowel Crohn disease. The ROC AUCs for polypoid pattern and homogeneous mural enhancement in the detection of intestinal Behçet disease were 0.806 and 0.779. The accuracy of MRE was 70.00% (95% CI, 50.60-85.27%); sensitivity, 57.14% (95% CI, 34.02-78.18%), and specificity, 100% (95% CI, 66.37-100%). CONCLUSION: MRE has potential for use as a radiation-free alternative for clarifying the cause of nonspecific gastrointestinal symptoms in patients with known Behçet disease. However, additional studies are needed to determine the actual value of MRE in patients with Behçet disease and to validate the clinical usefulness of the technique in the detection of unknown intestinal Behçet disease.

Lymphocytic hypophysitis associated with Behcet's disease: A case report and review of the literature.

Lymphocytic hypophysitis (LH) is a rare inflammatory disorder involving the pituitary gland, often with other autoimmune diseases combined. The coexistence of LH and Behçet\'s disease (BD) is a rare combination and only one case was reported in the previous literatures. A 50-year-old man was admitted into Sir Run Run Shaw Hospital presented with frontal headache and fatigue which had lasted for four months. Endocrinological inspection indicated anterior pituitary dysfunction. Magnetic resonance imaging (MRI) revealed homogeneous pituitary enlargement and thickened pituitary stalk. Administration of glucocorticoids could effectively relieve headache and reduced pituitary mass volume. Oral aphthosis, skin lesions and positive pathergy tests were recognized later, which were characteristic features of BD. Although the diagnosis of BD is mainly dependent on clinical manifestations, PT is still a useful diagnostic tool 0f high specificityfor BD. And this male patient was diagnosed with both LH and BD afterwards. Then he was treated with cyclophosphamide and medium doses of methylprednisolone and remained in good conditions at the follow-up. LH and BD might share a common underlying autoimmune pathogenesis. The presentation of endocrinologic disturbances such as anterior pituitary dysfunction with typical features of skin lesions should prompt further investigation of possible comorbid autoimmune disease involving multiple organ systems. Early diagnosis and close monitoring are vitally important to ensure a stable endocrinologic status.

Hemoptisis como manifestación inicial de la enfermedad de Behçet: casos clínicos / Hemoptysis as the initial manifestation of Behçet's disease: report of two cases

Arterial involvement in Behçet's disease (BD) is less common than venous lesions. The most commonly affected arteries are: the aorta, lower extremity arteries, mesenteric, femoral, coronary, renal, subclavian and pulmonary arteries. The rupture of pulmonary arteries is the main cause of death of patients with EB and the presence of aneurysms is a bad prognostic factor. We report two patients with arterial involvement in BD. A 14 years old male presenting with hemoptysis lasting three days. A chest computed tomography showed an aneurismal dilatation of the right interlobar artery, bilateral intramural thrombi and alveolar hemorrhage. A right lobar pulmonary resection was performed but hemoptysis recurred. Suspecting a BD, prednisone was started and hemoptysis subsided. A 42 years old male presenting with chest pain and hemoptysis. A chest CT scan showed thrombi in pulmonary veins and anticoagulant therapy was started. Two months later he was admitted again due to a massive hemoptysis. The CT scan showed aneurisms in pulmonary arteries, mural thrombi and pulmonary infarction. With the diagnosis of BD, methylprednisolone, followed by prednisone and cyclophosphamide were used, with a good clinical response.

Aortic Regurgitation Presenting with Recurrent Detachment of a Prosthetic Valve, as the First Presenting Symptom of Cardiovascular Behçet's Disease.

A 33-year-old man with severe aortic regurgitation underwent initial aortic valve replacement (AVR). During the 2 years after AVR, 3 reoperations for prosthetic valve detachment were required. During hospitalization, he had no typical clinical findings, with the exception of a persistent inflammatory reaction; a pseudo-aneurysm around the Bentall graft developed 27 days after the 4th operation. This unique clinical course suggested the possibility of Behçet's disease. In the 8 years of follow-up after the administration of prednisolone, the pseudo-aneurysm did not become enlarged and the detachment of the prosthetic valve was not observed. We herein present a case of cardiovascular Behçet's disease, with a review of the literature.

One year in review 2017: Behçet's syndrome.

A meta-analysis showed that methodological differences in prevalence studies such as a sample survey design or census design may be responsible for some of the variance in BS prevalence reported across countries, in addition to a true geographic variation. Efforts towards developing a data driven core set of outcome measures for clinical trials is continuing. Multimodal imaging using color fundus photography, fluorescein angiography, and optical coherence tomography is essential in visualising diagnostic features, detecting structural changes, and monitoring disease activity and response to treatment in Behçet's uveitis. Haemoptysis could also be due to bronchial artery enlargement in BS patients with pulmonary artery involvement and can be effectively treated with embolisation. Recent studies shed light on the link between immune system and thrombosis: fibrin clots seemed to be structurally different and plasmin resistant in BS. Newer genetic associations using immunochip were determined, but HLA-B51 is still the principal genetic link. Various studies on micro-RNA's, important molecules of immune regulation were published and discussed. Anti-TNF agents are still the key biologics for the treatment of various manifestations of BS. Two Phase III trials enrolling a small number of BS patients have shown the efficacy of adalimumab in the treatment of non-infectious, non-anterior uveitis. Interferon-alpha was found to induce long-lasting drug free remissions in a retrospective study. Small observational studies with non-TNF biologics such as ustekinumab, anakinra and canakinumab report beneficial results which await confirmation with further studies.

CT Features of Vasculitides Based on the 2012 International Chapel Hill Consensus Conference Revised Classification.

Vasculitis, characterized by inflammation of vessel walls, is comprised of heterogeneous clinicopathological entities, and thus poses a diagnostic challenge. The most widely used approach for classifying vasculitides is based on the International Chapel Hill Consensus Conference (CHCC) nomenclature system. Based on the recently revised CHCC 2012, we propose computed tomography (CT) features of vasculitides and a differential diagnosis based on location and morphological characteristics. Finally, vasculitis mimics should be differentiated, because erroneous application of immunosuppressive drugs on vasculitis mimics may be ineffective, even deteriorating. This article presents the utility of CT in the diagnosis and differential diagnosis of vasculitides.