Retinal thickness profiles in patients with Behçet uveitis during remission / Perfis de espessura da retina em pacientes com uveíte de Behçet durante remissão

ABSTRACT Purpose: The purpose of this study was to evaluate the intraretinal layer thickness in the macular region and its correlation with the duration of uveitis and visual acuity in patients with Behçet uveitis. Methods: In this cross-sectional study, we included 93 eyes of 57 patients with Behçet uveitis and 100 eyes of 50 healthy individuals admitted to a tertiary center from January to September 2017. We performed macular measurements in all subjects via spectral domain-optical coherence tomography (SD-OCT) and divided the retina into layers using automated segmentation software on the SD-OCT device. We then compared layer thicknesses between the patient and control groups and evaluated the correlation between OCT parameters and the duration of uveitis and visual acuity in the patient group. Results: Our records show a mean age of 37.9 ± 10.8 (18-64) years and 37.7 ± 12.2 (21-61) years in the patient and control groups (p=0.821), respectively. Meanwhile, data reveal a mean duration of uveitis of 6.9 ± 4.7 (1-20) years. We found a reduction in the total outer layer thickness in the patient group (p<0.001). However, we did not find a statistically significant difference in the inner retinal layers except in the inner nuclear layer. The duration of uveitis negatively correlated with the outer retinal layer's thickness (correlation coefficient = -0.250). On the other hand, visual acuity positively correlated with the central macular, the total inner layer, and the outer retinal layer thicknesses (correlation coefficients: 0.194, 0.154, and 0.364, respectively). However, the inner nuclear layer negatively correlated with visual acuity. Conclusions: Using retinal segmentation via SD-OCT for follow-ups can help estimate visual loss in patients with Behçet uveitis, which can cause significant changes in intraretinal layers in the macular region.

RESUMO Objetivo: Avaliar a espessura das camadas intraretinianas na região macular e sua relação com a duração da uveíte e acuidade visual em pacientes com uveíte de Behçet. Métodos: Este estudo transversal incluiu 93 olhos de 57 pacientes com uveíte de Behçet e 100 olhos de 50 indivíduos saudáveis que foram admitidos em um hospital terciário entre janeiro de 2017 e setembro de 2017. As medições maculares foram realizadas com tomografia de coerência óptica de domínio espectral (SD-OCT) em todos os pacientes. A retina foi dividida em camadas usando software de segmentação automatizado no dispositivo SD-OCT. As espessuras da camada foram comparadas entre os pacientes e os grupos controle. No grupo de pacientes, foi avaliada a correlação entre os parâmetros obtidos na OCT e a duração da uveíte e acuidade visual. Resultados: A média de idade foi de 37,9 ± 10,8 (18-64) no grupo de pacientes e 37,7 ± 12,2 (21-61) no grupo controle (p=0,821). A duração média da uveíte foi de 6,9 ± 4,7 (1-20) anos. A espessura total das camadas externas no grupo de pacientes foi reduzida (p<0,001). Uma diferença estatisticamente significativa não foi encontrada nas camadas internas da retina, exceto na camada nuclear interna. Uma correlação negativa foi detectada entre a duração da uveíte e a espessura da camada externa da retina (coeficiente de correlação = -0,250). Uma correlação positiva significativa foi detectada entre a acuidade visual e a espessura macular central bem como a espessura total das camadas internas e externas da retina (coeficientes de correlação 0,194; 0,154 e 0,364, respectivamente). A camada nuclear interna foi negativamente correlacionada com a acuidade visual. Conclusões: A uveíte de Behçet pode causar alterações significativas nas camadas intraretinianas na região macular. A segmentação da retina com SD-OCT pode ser útil para acompanhamentos e para estimar a perda visual em pacientes com uveíte de Behçet.

Clinical profile and management with immunosuppressants and biologics in Behcet’s uveitis: A cohort of 25 patients from a tertiary eye care center in South India

Purpose: To report the clinical profile of Behcet’s disease and its management with immunosuppressants and biologics in a cohort of 25 patients from a tertiary eye care center in South India. Methods: This was a retrospective, observational study. Records of 45 eyes of 25 patients between January 2016 and December 2021 were retrieved from the hospital database. Complete ophthalmic evaluation and systemic examination by the rheumatologist with appropriate investigations had been done. Results were analyzed using Statistical Package for the Social Sciences (SPSS) software. Results: Males (19, 76%) were found to be more affected than females (6, 24%). Mean age of presentation was 27.68 ± 11.08 years. Twenty patients had bilateral involvement (80%), and unilateral involvement was seen in five patients (20%). Seven eyes of four patients (16%) had isolated anterior uveitis, out of which one patient had unilateral and three patients had bilateral involvement. Twenty?six eyes of 16 patients (64%) had posterior uveitis, out of which six patients had unilateral and 10 had bilateral involvement. Twelve eyes of seven patients (28%) had panuveitis, out of which two patients had unilateral and five had bilateral involvement. Hypopyon was seen in five eyes (11.1%) and posterior synechiae in seven eyes (15.55%). Posterior segment findings included vitritis (24.44%), vasculitis (17.78%), retinitis (17.78%), disc hyperemia (11.11%), and disc pallor (8.89%). Steroids alone were given in five patients (20%) and intravenous methylprednisolone (IVMP) was given in four patients (16%). Immunosuppressive agents along with steroids were given in 20 patients (80%), of which azathioprine alone was given in seven patients (28%), cyclosporin alone was given in two patients (8%), mycophenolate mofetil alone was given in three patients (12%), combination of azathioprine and cyclosporin was given in six patients (24%), and combination of methotrexate and mycophenolate mofetil was given in one patient (4%). Biologics were given in 10 patients (40%) – adalimumab in seven patients (28%) and infliximab in three patients (12%). Conclusion: Behcet’s disease is an uncommon uveitis in India. Addition of immunosuppressants and biologics to conventional steroid therapy gives better visual outcomes.

Cardiac Thrombosis in Behçet's Disease: A Diagnostic Challenge

Intracardiac masses have several etiologies: tumor, infectious, thrombotic. They constitute a diagnostic challenge for the clinician. Behçet's disease is a chronic multisystemic vasculitis evolving by relapses. Intracadiac thrombosis in this disease deserves special attention because of its life-threatening nature. They often appear in the early stages of the disease, and the most frequent location is the right heart chambers. We present the case of a 23-year-old patient with an isolated mass in the right ventricle revealing an initial manifestation of Behçet's disease. The presented clinical case highlights the importance of early recognition of Behçet's disease, as well as the awareness of including this disease in the differential diagnosis of intracardiac masses in young adults.

Abordaje de la enfermedad de Behçet vulvar: comunicación de caso

La enfermedad de Behçet (EB) es un trastorno inflamatorio, multisistémico, recidivante y remitente de etiología desconocida. Una característica clave de la enfermedad es la ulceración orogenital que provoca una morbilidad considerable con gran impacto en la calidad de vida de los pacientes. Su manejo médico consiste en un esquema con colchicina, esteroides o inmunomoduladores. Comunicamos el caso de una paciente con lesiones genitales quien consultó en múltiples ocasiones, recibiendo manejo antibiótico e incluso quirúrgico, con respuesta refractaria a estas intervenciones. Posteriormente, por las características de las lesiones y el comportamiento clínico se sospechó enfermedad de Behçet y se instauró manejo médico con esteroides orales y curaciones con fitoestimulina. Hubo resolución de las úlceras genitales y posteriormente la paciente fue llevada a cirugía para corrección de las sinequias vulvares.

Behçet's disease (BD) is an inflammatory, multisystemic, relapsing-remitting disorder of unknown etiology. A key feature of the disease is orogenital ulceration that causes considerable morbidity with great impact on patient's quality of life. Its medical management consists of a scheme with colchicine, steroids or immunomodulators. We report the case of a patient with genital lesions who consulted on multiple occasions, receiving antibiotics and even surgical management, with refractory response to these interventions. Subsequently, due to the characteristics of the lesion and clinical behavior, Behçet's disease was suspected and medical management with oral steroids and cures with phytostimulin was initiated. The genital ulcers were resolved, and the patient was subsequently taken to surgery for correction of the vulvar synechiae.

Dilated Cardiomyopathy with Biventricular Thrombi: A Severe Manifestation of Behcet Disease

Behcet’s disease is a systemic vasculitis of the vessels for all calibers, touching arterial and venous territories. The causes of disease are unknow. BD reaches young age subjects from 10 to 45 years and affects both men and women. BD is ubiquitous but more frequent in patients from Mediterranean basin, the middle East and Asia. The diagnosis of BD is essentially clinical. The diagnostic criteria make it possible to carry the diagnosis with good sensitivity and specifity. BD evolves by recurrent inflammatory attack. BD can affect all of the organs; cardiacs manifestations are dominated by intracardiac thrombosis, the damage of three tunics, coronaryarteritis with or without myocardial infarction, coronaries aneurysms and endomyocardial fibrosis. The vascular manifestations are dominated by arterial or venous thrombosis. The presence of dilated cardiomyopathy with reduced left ventricular ejection fraction is rare. It can be explained by ischemic or inflammatory origin by cytokines. We report a case of young woman aged of 33 years to the history of 3 episodes of bipolar aphtae which presented dilated cardiomyopathy with reduced left ventricular function, biventricular thrombosis, bilateral distal pulmonary embolism with pulmonary infarction.

Lower Digestive Tract Bleeding in a Patient with Behçet's Disease: A Case Report

Behçet's disease is a chronic, multisystemic, and relapsing inflammatory pathology that frequently manifests with oral and genital ulcers and ocular and skin lesions. It rarely exhibits gastrointestinal involvement, which varies depending on the affected gastrointestinal segment; these have in common the predominance of ulcerated lesions and, consequently, a greater risk of bleeding from the digestive tract. A clinical case of a 28-year-old female patient who consulted for a clinical picture of melenic stools and oral ulcers is described. As a crucial clinical history, she had been diagnosed with Behçet's disease since adolescence, associated with severe gastrointestinal complications. An esophagogastroduodenoscopy was performed with findings of antral erythematous gastropathy and a colonoscopy with a report of ulcerated ileitis. Treatment with azathioprine and corticosteroids was indicated, significantly improving the clinical picture.

La enfermedad de Behçet es una patología inflamatoria crónica, multisistémica y recidivante que se manifiesta frecuentemente con úlceras orales y genitales, y lesiones oculares y cutáneas. En raras ocasiones presenta compromiso gastrointestinal y este varía dependiendo del segmento gastrointestinal afectado; estas enfermedades tienen en común el predominio de lesiones ulceradas y, consecuentemente, un mayor riesgo de sangrado de las vías digestivas. Se describe un caso clínico de una paciente femenina de 28 años que consultó por un cuadro clínico de deposiciones melénicas y úlceras orales. Como antecedente clínico de importancia presentó diagnóstico de enfermedad de Behçet desde la adolescencia asociado a complicaciones gastrointestinales graves. Se realizó una esofagogastroduodenoscopia con hallazgos de gastropatía eritematosa antral y una colonoscopia con reporte de ileítis ulcerada. Se indicó el tratamiento con azatioprina y corticoides con una mejoría significativa del cuadro clínico.

The clinical characteristics and surgical prognosis of Beh?et′s disease with heart valve involvement and literature review / 中华风湿病学杂志

Objective:To investigate the clinical characteristics and treatment of Beh?et′s disease complicated with cardiac valve involvement.Methods:We searched the wanfang medical database and Medline database to reviewed the domestic and foreign literature reports on cardiac Beh?et′s disease and analyzed their clinical features and therapeutic strategies. Chi-squared test was used for data analysis.Results:It was shown that Beh?et′s disease with cardiac valve involvement mainly affect men. The male to female ratio was 3.86∶1 in China and 2.50∶1 in foreign patients( χ2=1.32, P=0.251). The preoperative diagnosis rate was not high(60.3% in China, 57.1% abroad) ( χ2=0.13, P=0.716). Aortic valve and perivalvular lesions were the most common involved sites, of which aortic regurgitation was the most frequenty occurred, followed by mitral valve lesions. Glucocorticoids was still the main means treatment for medical(93/235 in China, 28/420 abroad), cyclophosphamide was more widely used in China(28/235), azathioprine was more widely used in foreign countries (12/42). Aortic replacement (AVR) was the mainly surgical approach, followed by artificial aortic valve replacement and left ventricular outflow tract plasty (Bentall).The incidence of postoperative perivalvular leakage or valve prolapse was higher with AVR than with Bentall(AVR 76.3%/Bentall 21.8% at home, χ2=32.60, P<0.001, AVR 71.4%/Bentall 0 abroad, χ2=13.84, P<0.001). Conclusions:Cardiac valve involvement is a severe complication of Beh?et′s disease. Heart involvement are more common, and the preoperative diagnosis rate is lower in China. The incidence of perivalve leakage (PVL) or valve prolapse (PD) after operation is higher with AVR than with Bentall surgery.The Bentall operation could improve prognosis and the postoperative complications abroad are lower than domestic.

Trastornos inmunitarios en paciente pediátrico con enfermedad de Behcet / Inmune disorders in a pediatric patient with Behcet's disease

Introducción: La enfermedad de Behcet es un proceso autoinflamatorio crónico que afecta arterias y venas de todos los calibres. Participan factores genéticos, microbianos e inmunológicos. Cursa con úlceras orales, genitales e inflamación ocular. Objetivo: Describir los trastornos inmunitarios en un paciente pediátrico con enfermedad de Behcet. Presentación del caso: Paciente masculino de 16 años de edad, con diagnóstico clínico de enfermedad de Behcet con marcadores serológicos de autoinmunidad negativa. Las inmunoglobulinas séricas exhibieron valores normales. Las subpoblaciones linfocitarias T CD3+CD4+ y CD3+CD8+ estuvieron elevadas. Las células B totales mostraron valores porcentuales normales, sin embargo, la subpoblación B CD5+CD19+ se encontró por encima de los valores referenciales. El paciente respondió favorablemente en relación con el curso de las infecciones a la terapia con inmunoestimulantes. Conclusiones: El tratamiento para la enfermedad de Behcet debe ser individualizado. Se debe tener en cuenta la gravedad del órgano afectado y el índice de recurrencias. Se requiere de un grupo interdisciplinario para llegar al adecuado control de la enfermedad.

Introduction: Behcet's disease is a chronic autoinflammatory process, of low frequency and unknown etiology that affects arteries and veins of all sizes, where genetic, microbial and immunological factors participate. It presents with oral and genital ulcers and ocular inflammation. Objective: To evaluate the immune disorders in a pediatric patient with Behcet's disease. Case presentation: A 16-year-old male patient with a clinical diagnosis of Behcet's disease with negative autoimmunity serological markers. Immunoglobulins exhibited normal values. CD3+CD4+ and CD3+CD8+T lymphocyte subpopulations were elevated. The B cells showed normal percentage values, however the CD5+CD19+ subpopulation B was found to be above the reference values. The patient responded favorably in relation to the course of the infections, to the therapy with immune stimulants. Conclusions: Treatment must be individualized, taking into account the severity of the affected organ and the recurrence rate. An interdisciplinary group is required to achieve adequate control of the disease.

The roles of immune cells in Behçet's disease

Abstract Behçet's disease (BD) is a systemic vasculitis that can affect multiple systems, including the skin, mucous membranes, joints, eyes, gastrointestinal and nervous. However, the pathogenesis of BD remains unclear, and it is believed that immune-inflammatory reactions play a crucial role in its development. Immune cells are a critical component of this process and contribute to the onset and progression of BD. By regulating the function of these immune cells, effective control over the occurrence and development of BD can be achieved, particularly with regards to monocyte activation and aggregation, macrophage differentiation and polarization, as well as T cell subset differentiation. This review provides a brief overview of immune cells and their role in regulating BD progression, which may serve as a theoretical foundation for preventing and treating this disease.

Clinical characteristic of aortitis in cardiac surgery in a single center / 中国胸心血管外科临床杂志

@#Objective    To review and analyze the clinical manifestations of common aortitis in cardiac surgery. Methods    We screened 41 552 patients who were hospitalized in the Department of Cardiac Surgery of Zhongshan Hospital, Fudan University from 2010 to 2020, analyzed the patients' clinical data, and classified according to the type of diseases. Then we summarized all their clinical manifestations. Results    In our center 145 patients were operated for aortitis diseases, including 75 males and 70 females, with the age of 24-76 (45.6±11.3) years. There were 61 patients of Takayasu's arteritis, 51 patients of Behcet's disease, 8 patients of syphilitic aortitis, 8 patients of systemic lupus erythematosus, 2 patients of Kawasaki disease, 4 patients of ankylosing spondylitis, 10 patients of dry syndrome, and 1 patient of scleroderma. Conclusion    Aortitis is not uncommon in cardiac surgery, and awareness of the disease should be enhanced. So that we can distinguish various types of aortitis and to make proper management to improve patients' prognosis.

The experience in reoperation of Behcet's disease patient with perivalvular leakage after aortic valve replacement / 中华胸心血管外科杂志

Objective:To introduce the application of sleeve reconstruction with bovine pericardium in the reoperation of Behcet's disease with advanced aortic annulus invasion, and to report our experience and the early outcomes.Methods:From August 2012 to May 2022, 19 patients with Behcet's disease developed perivalvular leakage after previous surgery underwent aortic root replacement. They were analyzed retrospectively. Fifteen out of 19 cases underwent conventional aortic root replacement, 4 cases with advanced aortic annulus invasion underwent sleeve reconstruction with bovine pericardium. The medical records were reviewed. Demographic and perioperative data were collected, which included the aortic crossclamp time, intraoperative RBC consumption, length of ICU stay, and major postoperative complications.Postoperative follow-up was accomplished through telephone visit.Results:The median age was 39(36, 42) years old. Five were female. The median interval of diagnosis of perivalvular leak from index surgery was 5(3, 14)months. There was no perioperative death. Fifteen patients underwent conventional aortic root replacement. One patient had intraoperative ECMO impant due to low cardiac output as well as another one had IABP support. CABG was performed in 5 patients. One patient underwent permanent pacemaker implantation after surgery. The median follow-up time for the 15 patients was 55.00(29.25, 71.25)months. Two patients were lost during follow-up. One patient died of infection 3 years after surgery. One patient developed perivalvular leak. Two patient developed distal anastomotic pseudoaneurysm which need reintervention. The median follow-up time for the 4 patients with sleeve reconstruction was 5.5(5, 11.25)months. One patient underwent tracheotomy. Perivalvular leakage and pseudoaneurysm were not appreciated. Four patients were in NYHA class Ⅰ-Ⅱ.Conclusion:The sleeve reconstruction with bovine pericardium is safe and effective in reoperation treating patients with Behcet's disease and advanced aortic annulus invasion.

Intestinal Behcet’s : A Rare Presentation of Behcet’s Disease

We herein present a case of 48 years old female patient presented with fever, bloody diarrhea followed by palpable purpuric rash over the body along with recurrent oral and genital ulceration. These were associated with history of symmetric polyarthralgia. On examination moderate anemia, signs of anterior uveitis were found. In blood parameters thrombocytopenia along with elevated Erythrocyte Sedimentation Rate, C-Reactive Protein were noted. On further investigations the serological tests were found to be negative for Dengue, Chikungunya, HIV, HBV, HCV. Complement C3 found to be low. Colonoscopic biopsy is diagnostic of Indeterminate Crohn’s Disease with IgA, G, M, C3, Fibrinogen immunostaining in skin biopsy. ANA, P-ANCA, C-ANCA were found to be nonreactive for the patient. All of the above mentioned points were pointing towards Behcet’s disease. For confirmation, Anti Saccharomyces Cerevisae Antibody was found to be positive . Skin pathergy test was positive. So, we diagnosed this case as behcet’s disease.

Aneurismas de las arterias pulmonares en relación al síndrome de Hughes Stovin / Aenurysms of the pulmonary arteries in relation to Hughes Stovin syndrome

El síndrome de Hughes Stovin es una vasculitis de pequeños y grandes vasos, más frecuente en hombres jóvenes. Se manifiesta con trombosis y aneurismas. Inicia con tromboflebitis que evoluciona a aneurismas con eventual desenlace fatal por ruptura de los mismos. Algunos autores consideran el síndrome de Hughes Stovin como una variante de la enfermedad de Behçet. El diagnóstico es clínico y el tratamiento de primera línea son los corticoides y la ciclofosfamida. Frente a un caso de trombosis, la anticoagulación es controversial por el riesgo de sangrado. Presentamos el caso de un varón joven con cuadro clínico compatible en el cual se descartaron otras patologías con requerimiento de tratamiento quirúrgico por mala evolución clínica.

Hughes Stovin syndrome is characterized by small and big vessels vasculitis, more commonly presented in young men. Initially it manifests with thrombophlebitis and aneurysms which can cause fatal bleed due to rupture. Some authors consider Hughes Stovin syndrome as a variant of Behcet's disease. Diagnosis is made by clinical findings and the first line treatment are corticosteroids and cyclophosphamide. Anticoagulation is controversial due to the increased risk of hemorrhage.

Manifestaciones gastrointestinales de la nfermedad de Behçet / Gastrointestinal manifestations of Behçet´s disease

Resumen La Enfermedad de Behçet (EB), es una entidad poco común, idiopática, de naturaleza crónica y recurrente con afectación multisistémica. Entre ellos, el tracto gastrointestinal ha suscitado especial interés, al asociarse a mayor morbimortalidad y dado sus presentaciones heterogéneas puede simular otras enfermedades gastrointestinales y ser un gran reto diagnóstico para el gastroenterólogo. Por lo tanto, presentamos una revisión narrativa donde se pretenderá describir las manifestaciones gastrointestinales de la EB y sus diagnósticos diferenciales.

Abstract Behçet's Disease (BD) is a rare, idiopathic, chronic and relapsing entity; characterized by multisystem involvement. The gastrointestinal tract has aroused special interest, as it is associated with higher morbidity and mortality and given its heterogeneous presentations, being able to simulate other gastrointestinal pathologies, becoming a great diagnostic challenge for gastroenterologists. Therefore, we present a narrative review, where we intend to describe the possible gastrointestinal manifestations of BD and its differentials.

Manejo anestésico de un paciente con enfermedad de Behçet / Anesthetic management of a patient with Behçet´s Disease Sobre los autores:

RESUMEN Introducción: La enfermedad de Behçet es una enfermedad inflamatoria crónica, recurrente, multisistémica, de etiología desconocida, caracterizada por úlceras orales y genitales recurrentes, inflamación ocular, lesiones cutáneas, artritis, afecciones neurológicas, pulmonares, gastrointestinales y vasculitis sistémica. Objetivo: Describir el manejo anestésico en un paciente portador de enfermedad de Behçet. Presentación del caso: Se reporta el caso de un paciente de 52 años de edad con antecedentes patológicos personales de enfermedad de Behçet que recibe anestesia general para exéresis de adenopatía cervical izquierda metastásica de un carcinoma primario oculto. Conclusiones: El mantenimiento del tratamiento con esteroides, el uso de nadroparina cálcica junto a otras medidas preventivas de la trombosis venosa profunda, el manejo cuidadoso de la vía aérea, la protección ocular y la articular, así como de los puntos de presión y la prevención de la patergia son elementos fundamentales en el manejo de estos pacientes.

ABSTRACT Introduction: Behçet's disease is a chronic, recurrent, multisystemic inflammatory disease of unknown etiology characterized by recurrent oral and genital ulcers, ocular inflammation, skin lesions, arthritis, neurological, pulmonary and gastrointestinal conditions, as well as systemic vasculitis. Objective: To describe the anesthetic management of a patient with Behçet's disease. Case presentation: The case is reported of a 52-year-old male patient with an individual history of Behçet's disease, who receives general anesthesia for removal of left cervical metastatic adenopathy from a hidden primary carcinoma. Conclusions: Keeping the steroid therapy, using calcium nadroparin, together with other measures for preventing deep vein thrombosis; careful airway management, eye and joint protection, as well as attention to pressure points and pathergy prevention are fundamental elements for the management of these patients.

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

ABSTRACT 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.

Cerebrovascular changes in neuro-Behcet's disease and neuropsychiatric lupus by transcranial Doppler ultrasonography and magnetic resonance imaging

ABSTRACT Introduction: Transcranial Doppler ultrasonography (TCD) is a technique that allows measurement of blood flow from the basal intracerebral vessels. It is relatively inexpensive, non-invasive, can be performed at the bedside, and allows monitoring in acute emergency settings and for prolonged periods with a high temporal resolution, making it ideal for studying the haemodynamics within the intracranial arteries in neuro-Behcet's disease (NBD) and neuro-psychiatric lupus (NPSLE). Our aim was to assess the cerebral haemodynamic patterns in patients with NBD and NPSLE using TCD, while brain lesions were examined using magnetic resonance imaging (MRI). Material and methods: Case-control prospective study of 30 neuro-Behcet's disease patients, 25 neuro-psychiatric lupus patients and 26 healthy age-matched volunteers. All patients and healthy controls were examined by TCD. Only the groups of patients underwent cranial magnetic resonance imaging (MRI). Results: Transcranial Doppler (TCD) values for middle cerebral artery (MCA), anterior cerebral artery (ACA), posterior cerebral artery (PCA), vertebral artery (VA) and basilar artery (BA) in NBD, NPSLE and control groups were measured. The results showed that there was a significant decrease in mean blood flow velocities in all the arteries examined in NBD and NPSLE patients. There was also a significant increase in the pulsatile index of PCA, VA and BA between NBD and NPSLE patients. The same results were obtained when comparing NBD versus controls. However, there was no significant difference between the NPSLE patients and the control group. The MRI lesions described were parenchymal lesions in 14 patients (46.7%), and vascular lesions in 4 patients (13.3%). Vascular lesions co-existed with parenchymal lesions (mixed lesion). Parenchymal lesions were in white matter (40%), thalamus (26.7%), brain stem (26.7%) and cerebellum (20%). While, in NPSLE, 23 patients were normal (92%) and only two patients had a vascular lesion (8%). Conclusion: There was a significant decrease in mean blood flow and a significant increase in the pulsatile index among both NBD and NPSLE patients, according to the TCD values.

O intestino delgado é amplamente afetado na doença de Behçet: um acompanhamento de longo prazo dos sintomas gastrointestinais / Small bowel is largely affected in behçet's disease: a long-term follow-up of gastrointestinal symptoms

ABSTRACT Background Behçet's disease is a rare immune-mediated disorder that can affect the gastrointestinal tract. The prevalence and extension of small bowel involvement is largely unknown. Objective The aim of this study was to describe the small bowel lesions diagnosed by double-balloon enteroscopy (DBE) and to verify if these findings were associated to the presence of gastrointestinal symptoms and disease activity after long-term follow-up. Methods This study included 19 Behçet's disease patients who underwent DBE. After a mean follow-up of 15 years the endoscopic findings were associated to the presence of gastrointestinal symptoms, disease activity and current therapy through collection of electronic medical records. Results A total of 63.2% patients were female and the mean age was 37 years at the time of DBE. Mean disease duration at baseline was 24 years. 11 patients had no gastrointestinal symptoms and eight patients presented either abdominal pain, gastrointestinal bleeding or diarrhea. The average procedure time was 1 hour and 30 minutes and the ileum was achieved in all patients but one. Small bowel ulcers were diagnosed in 78.9%, with 63.1% of jejunal involvement. Two patients presented only small bowel edema and two were normal by DBE. Eight patients had concomitant gastric ulcers. Gastrointestinal symptoms prior to DBE were present in 36.8% of the patients and, after follow-up, all of them persisted with some of the symptoms. Bleeding was reported by three patients at baseline and persisted in only one patient. The frequency of treatment with steroids and immunomodulators was 31.6% and 57.9% at baseline, respectively, and 21% in both at the end of the follow-up. No patient was treated with biologics at the time of the DBE procedure and the current rate of biologic use is 21%. Conclusion Small bowel involvement in Behçet's disease was frequently demonstrated by DBE even in asymptomatic patients. Understanding clinical evolution of the disease over the years and the impact of such diagnosis still represents a challenge, possibly with the need for novel treatment.

RESUMO Contexto A doença de Behçet é uma doença imunomediada rara que pode afetar o trato gastrointestinal. A prevalência e extensão do envolvimento do intestino delgado é desconhecida. Objetivo O objetivo deste estudo foi descrever as lesões do intestino delgado diagnosticadas por enteroscopia de duplo balão (EDB) e verificar se esses achados estavam associados à presença de sintomas gastrointestinais e atividade da doença após seguimento de longo prazo. Métodos Este estudo incluiu 19 pacientes com doença de Behçet que foram submetidos a EDB. Após seguimento médio de 15 anos, os achados endoscópicos foram associados à presença de sintomas gastrointestinais, atividade da doença e terapia atual por meio de coleta de prontuário eletrônico. Resultados Um total de 63,2% dos pacientes eram do sexo feminino e a média de idade era de 37 anos no momento da EDB. A duração média da doença no início do estudo foi de 24 anos. 11 pacientes não apresentaram sintomas gastrointestinais e oito pacientes apresentaram dor abdominal, sangramento gastrointestinal ou diarréia. O tempo médio do procedimento foi de 1 hora e 30 minutos e o íleo foi atingido em todos os pacientes, exceto em um. Úlceras de intestino delgado foram diagnosticadas em 78,9%, sendo 63,1% de acometimento jejunal. Dois pacientes apresentaram apenas edema de intestino delgado e dois apresentaram EDB normais. Oito pacientes tinham úlceras gástricas concomitantes. Sintomas gastrointestinais prévios à EDB estavam presentes em 36,8% dos pacientes e, após o seguimento, todos persistiram com alguns dos sintomas. Sangramento foi relatado por três pacientes no início do estudo e persistiu em apenas um paciente. A frequência de tratamento com esteroides e imunomoduladores foi de 31,6% e 57,9% no início do estudo, respectivamente, e 21% em ambos ao final do seguimento. Nenhum paciente foi tratado com biológicos no momento da EDB e a taxa atual de uso de biológicos é de 21%. Conclusão O envolvimento do intestino delgado na doença de Behçet foi frequentemente demonstrado por EDB mesmo em pacientes assintomáticos. Compreender a evolução clínica da doença ao longo dos anos e o impacto de tal diagnóstico ainda representa um desafio, possivelmente com a necessidade de novos tratamentos.

Clinical characteristics, diagnosis and evaluation of intestinal Behcet′s disease / 中华全科医师杂志

Behcet′s disease is a chronic systemic vasculitis with unknown etiology that can involve multiple organs. Behcet′s disease with predominant gastrointestinal manifestations is diagnosed with intestinal Behcet′s disease. Severe complications such as gastrointestinal massive hemorrhage, perforation and obstruction may occur in intestinal Behcet′s disease. If not treated in time, they are associated with significant morbidity and mortality. Here, we will review the lesion distribution, clinical manifestations, diagnosis and disease activity of intestinal Behcet′s disease for better understanding of clinicians.

Tratamento endovascular na doença de Behçet: uma revisão integrativa / Endovascular treatment in Behçet's disease: an integrative review

Resumo A doença de Behçet (DB) é caracterizada por ser uma doença rara, crônica, recidivante, inflamatória e multissistêmica. Quanto ao diagnóstico, não existe nenhum teste universalmente descrito; logo, ele é feito de maneira clínica, por meio da presença da sua tríade clássica de sinais: úlceras orais, úlceras genitais e uveíte (inflamação do trato uveal do olho). O objetivo deste estudo foi avaliar a importância do tratamento endovascular na doença de Behçet, sendo que a revisão de literatura para obter essa resposta foi realizada a partir de 30 artigos publicados entre 2002 e 2021. A DB acomete tanto o sistema venoso quanto o arterial. A ruptura de aneurismas é a principal causa de morte e necessita de tratamento, podendo ser clínico, cirúrgico aberto ou por via endovascular. A cirurgia endovascular vem ganhando espaço no tratamento cirúrgico nos casos de aneurismas arteriais, inclusive nos relacionados com DB, apesar de a terapia de escolha ainda ser controversa.

Abstract Behçet's Disease (BD) is a rare, chronic, relapsing, inflammatory, and multisystemic disease. There is no universally described test for confirming diagnosis, so it is made clinically, on the basis of its classic triad of signs: oral ulcers, genital ulcers, and uveitis (inflammation of the uveal tract of the eye). The aim of this study is to evaluate the importance of endovascular treatment in Behçet's disease. The literature review conducted to achieve this objective covered 30 articles published between 2002 and 2021. Behçet's Disease affects both the venous and arterial systems. Rupture of aneurysms is the main cause of death and thus requires treatment, which can be clinical, open surgical, or endovascular. Endovascular surgery has been gaining ground for surgical treatment of arterial aneurysms, including those related to BD, although the therapy of choice is still controversial.