BehÒ«et's disease: A diagnostic challenge

BehÒ«et's disease is a low prevalence disorder that is difficult to diagnose. Its forms of presentation are often confused with infectious processes that involve multiple treatments and prolonged hospital stays. This article presents the case of a 30-year-old man who consulted with non-specific symptoms that represented a diagnostic challenge for the medical team.

La enfermedad de BehÒ«et es una patología de baja prevalencia y difícil diagnóstico; sus formas clínicas de presentación suelen confundirse con procesos infecciosos que implican múltiples tratamientos y estancias hospitalarias prolongadas. Este artículo presenta el caso de un hombre de 30 arios quien consulta de manera repetitiva con síntomas inespecíficos, lo que representa un reto diagnóstico para el equipo médico.

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.

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.

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.

COVID-19 related acute genital ulcer: a case report

ABSTRACT Acute vulvar ulcer (Lipschütz's ulcer) is a rare lesion with local hyperimmunoreactivity triggered by infection, which is characterized by acute, painful, and necrotic ulcerations. This condition is usually found in non-sexually active adolescents, and it resolves spontaneously. We report a case of a 35-year-old woman who was diagnosed with COVID-19 who did not have severe symptoms, but had high levels of D-dimer for 9 days. The COVID-19 diagnosis was followed by the appearance of an acute, necrotic, extremely painful vulvar ulcer, although symptoms caused by COVID-19 had improved. We emphasize the importance of the differential diagnosis to exclude diseases such as Behçet's syndrome, Sexually Transmitted Infections, as well as the presence of viruses that generally trigger Lipschütz's ulcer, such as Epstein-Barr virus and cytomegalovirus. No treatment is usually necessary, however, in the present report due to the pain experienced by the patient, we successfully used oral prednisone.

Estudio inmunológico en paciente con síndrome de Behcet: reporte de un caso / immunological study in a patient with Behcet Syndrome: a case report

Introducción: El síndrome de Behcet, o enfermedad de Behcet, es un proceso autoinflamatorio crónico, poco frecuente, de etiología desconocida. Es una vasculitis que afecta arterias y venas de todos los calibres, con alteración de la función endotelial, que se expresa clínicamente con lesiones orgánicas variadas. En su fisiopatogenia intervienen factores genéticos, microbianos e inmunológicos. Los síntomas más comunes son las úlceras orales y genitales, inflamaciones oculares (uveítis, retinitis e iritis), lesiones de piel y artritis. Objetivo: Evaluar diversos marcadores de la respuesta inmune en paciente con síndrome de Behcet. Presentación del caso: Paciente masculino. 39 años de edad, con diagnóstico clínico de enfermedad de Behcet con reactantes de fase aguda y marcadores serológicos de autoinmunidad negativa. Las subpoblaciones linfocitarias están dentro de los valores referenciales, sin evidencias de activación linfocitaria. La presencia de una doble población de linfocitos B y los antecedentes familiares, sugieren la existencia de una población de linfocitos B de autoreconocimiento y la posible presencia de factores genéticos, respectivamente. El paciente respondió favorablemente a la terapia con esteroides. Conclusiones: El estudio apoya el criterio de que, en condiciones basales, no se detectan marcadores humorales de autoinmunidad, alteraciones de los valores de las subpoblaciones linfocitarias, ni evidencias de activación linfocitaria, pero no se puede excluir la presencia de una población de linfocitos B de autoreconocimiento(AU)

Introduction: Behcet's syndrome, also known as Behcet's disease is a chronic autoinflammatory process of low frequency and unknown etiology. It's an all sizes arteries and veins affecting vasculitis that causes an alteration of endothelial function and is expressed clinically by organ damage at various levels. Its pathogenesis involves genetic, microbial and immunological factors. The most common symptoms are oral and genital ulcers, eye inflammation (uveitis, iritis and retinitis), skin lesions and arthritis. Objective: to evaluate several inmunological markers in a patient with Behcet syndrome. Case presentation: 39 years old masculine patientwith clinical diagnosis of Behcet disease with negative acute phase reactants and serological authoinmunity markers and lymphocyte populations within referential range, without evidences of lymphocyte activation. The presence of a double B lymphocyte population and familial background, suggest the presence of a self recognitionB lymphocyte population and the probable presence of genetic factors, respectively. There was a good response to steroids treatment. Conclusions: The study supports the idea that at baseline, not humoral autoimmunity markers, changes in the values of lymphocyte subpopulations, and evidence of lymphocyte activation is detected, but can not exclude the presence of a population of B lymphocytes self-recognition(AU)

Case for diagnosis. Ulcerated lesions: a diagnostic challenge in Behçet's syndrome

Abstract This study reports the clinical case of a 42-year-old patient with ulcerated lesions who was followed up by general practitioners with the diagnosis of recurrent cellulitis. However, when referred to the Dermatology division a diagnosis of Behçet's syndrome was established based on clinical criteria. Although there are defined clinical criteria for this syndrome, sometimes its diagnosis can be challenging, due to lack of knowledge of the disease and extremely heterogeneous clinical phenotype. The authors highlight the potential difficulties in establishing the diagnosis considering the multiple clinical findings during the investigation process, contributing to the risk of increased morbidity and mortality.

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

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

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

Perspectivas da saúde orofacial em portadores de doenças reumáticas: uma revisão da literatura / Perspectives of orofacial health in people with rheumatic diseases: a literature review

Este estudo objetivou verificar as principais manifestações bucofaciais que as doenças reumáticas podem causar. Foram selecionadas, assim, 05 doenças reumáticas: Artrite Reumatóide (AR), Síndrome de Sjogren (SS), Lúpus Eritematoso Sistêmico (LES), Esclerose Sistêmica (ES) e a Síndrome de Behçet (SB). Estas, por sua vez, foram escolhidas por apresentarem como sinais e sintomas clínicos problemas orofaciais. Foi elaborado, dessa maneira, uma revisão bibliográfica de trabalhos com vintênio de 2000 a 2020 nas seguintes bases: LILACS, MEDLINE e SCIELO. Propõe-se, portanto, a introdução de um Cirurgião-dentista na equipe multidisciplinar de Reumatologia para diagnosticar e tratar as especificidades bucofaciais que acometem os portadores de problemas reumáticos... (AU)

This study aimed to verify as main bucofacial manifestations that rheumatic diseases can cause. Thus, 05 rheumatic diseases were selected: Rheumatoid Arthritis (RA), Sjogren's Syndrome (SS), Systemic Lupus Erythematosus (SLE), Systemic Sclerosis (ES) and Behçet's Syndrome (SB). These, in turn, were chosenbecause they present as orofacial clinical signs and symptoms. In this way, a bibliographic review of works with twenty years from 2000 to 2020 was prepared on the following bases: LILACS, MEDLINE and SCIELO. Therefore, it is proposed to introduce a dental surgeon in the multidisciplinary team of Rheumatology to diagnose and treat as orofacial specificities that affect patients with rheumatic problems... (AU)

Enfermedad de Behçet frente a la enfermedad inflamatoria intestinal. Diferencias en la presentación clínica / Behçet's disease compared with inflammatory bowel disease. Differences in their clinical presentation

Resumen La enfermedad de Behçet es una enfermedad poco frecuente con un compromiso sistémico cuyas principales manifestaciones abarcan el sistema gastrointestinal, neurológico, vascular y articular, además de la piel y los ojos. Por su compromiso multisistémico, es frecuente confundirlo con otras entidades como la enfermedad inflamatoria intestinal, con la cual comparte gran parte de su presentación clínica, por lo que se describe un caso clínico y se aborda una revision narrativa desde el punto de vista epidemiológico de las manifestaciones clínicas con el fin de tener un mayor conocimiento para identificarla a tiempo.

Abstract Behçet's disease is a rare disease with systemic involvement whose main manifestations include the gastrointestinal, neurological, vascular, and articular systems, as well as the skin and eyes. Due to its multisystemic nature, it is often mistaken for other entities such as inflammatory bowel disease since they share multiple characteristics in their clinical presentation. A clinical case is described, and an epidemiological review of clinical manifestations is discussed to have better knowledge that allow identifying it on time.

Síndrome de Behçet ou úlcera de Lipschütz: desafio diagnóstico / Behçet's syndrome or Lipschütz ulcer: diagnostic challenge

A ocorrência de úlceras genitais em adolescentes e mulheres jovens tem impacto emocional para as pacientes e seus familiares, pela frequente associação com uma possível etiologia de transmissão sexual. Porém, úlcera de Lipschütz e síndrome de Behçet não têm etiologia infecciosa e devem ser lembradas como possíveis diagnósticos diferenciais. O diagnóstico dessas duas patologias é clínico e pode ser desafiador. Dessa forma, foi realizada uma revisão na literatura com o objetivo de comparar as duas entidades. A úlcera de Lipschütz é causada por uma vasculite local e caracteriza-se pelo surgimento súbito de úlceras na vulva ou vagina inferior. Já a doença de Behçet é causada por vasculite sistêmica, com episódios de remissão e exacerbação, que pode envolver quase todos os sistemas orgânicos. Em ambos os casos, é essencial o referenciamento para reumatologia. O tratamento objetiva suprimir exacerbações, controlar a dor e prevenir infecção secundária.(AU)

The occurrence of genital ulcers in adolescents and young women have an emotional impact for the patient and their families, due to the frequent association of its etiology with a sexually transmitted disease. However, Lipschütz ulcer and Behçet's syndrome do not have an infectious etiology and should be remembered as a possible differential diagnoses. As the diagnosis of these two pathologies is clinical and can be challenging, a review of literature was carried out. The objective of this review of literature was to compare both diseases. Lipschütz ulcer is caused by local vasculitis and is characterized by the sudden appearance of ulcers in the vulva or lower vagina. Behçet's syndrome is caused by systemic vasculitis, with episodes of remission and exacerbation, which can affect almost all organ systems. In both cases, referral to rheumatology is essential. Treatment aims to suppress exacerbations, control pain and prevent secondary infection.(AU)

Tratamento endovascular de aneurisma sacular aórtico associado à doença de Adamantiades-Behçet / Endovascular treatment of aortic saccular aneurysms associated with Adamantiades-Behçet disease

Resumo A doença de Adamantiades-Behçet é uma desordem multissistêmica que se apresenta classicamente com úlceras orais e genitais e envolvimento ocular, podendo o acometimento vascular ocorrer em até 38% dos casos. O envolvimento aórtico é uma das manifestações mais severas e está associado a altas taxas de mortalidade, ocorrendo em 1,5 a 2,7% dos casos. Relatamos um caso de aneurisma sacular de aorta abdominal em um paciente de 49 anos com doença de Adamantiades-Behçet complicada, tratada por correção endovascular.

Abstract Adamantiades-Behçet disease is a multisystemic disorder that classically presents with oral and genital ulcers and ocular involvement, with vascular involvement in up to 38% of cases. Aortic involvement is one of the most serious manifestations and is associated with high mortality rates, occurring in 1.5 to 2.7% of cases. We report a case of a saccular abdominal aorta aneurysm in a 49-year-old male patient with complicated Adamantiades-Behçet disease that was treated with endovascular repair.

Acometimento vascular na doença de Behçet: o processo imunopatológico / Vascular involvement in Behçet's disease: the immunopathological process

Resumo A doença de Behçet constitui uma forma rara de vasculite sistêmica, que acomete de pequenos a grandes vasos. É caracterizada por manifestações mucocutâneas, pulmonares, cardiovasculares, gastrointestinais e neurológicas. Sua apresentação clínica é bastante ampla, variando de casos mais brandos a casos graves, com acometimento multissistêmico, caracteristicamente com exacerbações e remissões. Suas causas ainda são desconhecidas; entretanto, há evidências genéticas, ambientais e imunológicas, como a associação com o alelo HLA-B51. Todas essas, em conjunto, apontam para um processo imunopatológico anormal, com ativação de células da imunidade inata e adaptativa, como as células natural killer, neutrófilos e células T, que geram padrões de respostas e citocinas específicos capazes de gerar mediadores que podem lesionar e inflamar o sistema vascular, resultando em oclusões venosas, arteriais e/ou formação de aneurismas.

Abstract Behçet's disease is a rare form of systemic vasculitis that affects small to large vessels. It is characterized by mucocutaneous, pulmonary, cardiovascular, gastrointestinal, and neurological manifestations. Its clinical presentation is quite wide, ranging from milder cases to severe cases, with multisystemic involvement, characteristically with exacerbations and remissions. Its etiopathogenesis is still unclear, although there is evidence of genetic, environmental, and immunological factors, such as the association with the HLA-B51 allele. In conjunction, all of these point to an abnormal immunopathological process, with activation of cells of innate and adaptive immunity, such as NK cells, neutrophils, and T cells, which generate specific response patterns and cytokines capable of generating mediators that can damage and inflame blood vessels, resulting in venous and arterial occlusions and/or aneurysm formation.

Enfermedad de Behcet: un reto diagnóstico en reumatología. Descripción de una serie de casos y revisión de la literatura / Behcet's disease: a diagnostic challenge in rheumatology. Description of a case series and a review of the literature

RESUMEN La enfermedad de Behcet es una entidad clínica autoinflamatoria, de etiología desconocida, generalmente con compromiso sistémico, con un patrón de exacerbación y remisión frecuente que se asocia a retraso en el diagnóstico. El diagnóstico de esta enfermedad es complejo, por esta razón presentamos 4 casos de pacientes con enfermedad de Behcet, que durante el abordaje clínico fueron consideradas otras enfermedades de naturaleza autoinmune. La revisión integrada de la historia clínica, la aparición de úlceras orales y genitales, así como el estudio de tipificación del complejo mayor de histocompatibilidad (HLA) permitieron diagnosticar la enfermedad de Behcet.

ABSTRACT Behcet disease is a rare autoinflammatory disorder of unknown aetiology and is characterised by systemic manifestations with an exacerbation-remission pattern, often associated with diagnostic delay. The diagnostic approach to this disease is complex. A report is given on four cases of patients fulfilling the diagnostic criteria for Behcet disease. Other autoimmune rheumatic diseases were considered in the clinical approach. A meticulous clinical evaluation, taking into consideration relapsing aphthous ulcers in oral mucosa and genitalia, and HLA typing allowed a proper diagnosis of Behcet disease to be made.

Síndrome de Behçet ­ os desafios do diagnóstico: uma revisão sistemática / Behçet Syndrome ­ the challenges of diagnosis: a systematic review

A Doença de Behçet (DB) é uma afecção entendida por ser inflamatória, multissistêmica,de causa ainda desconhecida, apresentando sintomas como úlceras orais, genitais, uveíte, lesões cutâneas e vasculites recorrentes, sendo mais frequente nos países que se estendem desde o Mediterrâneo até o extremo Oriente. Reconhecida atualmente como uma doença autoimune, a DB parece agregar elementos genéticos do portador e fatores ambientais desencadeantes. Para o estabelecimento de critérios internacionais para um diagnóstico, protocolos clínicos foram desenvolvidos para auxiliar a atividade da doença assim permitindo a padronização das pesquisas na área, ainda que não existam alterações laboratoriais ou histopatológicas definidas da doença. O diagnóstico depende de uma avaliação clínica criteriosa que, quando precoce, é determinante para o prognóstico.Neste artigo são revistos dados clínicos para o diagnóstico da Doença de Behçet.

Behçet's disease (DB) is an affection understood to be multisystemic inflammation of an as yet unknown cause, presenting symptoms such as oral ulcers, genitalia, uveitis, cutaneous lesions and recurrent vasculitis. It is more frequent in countries stretching from the Mediterranean to the Far East. Currently recognized as an autoimmune disease, DB appears to aggregate carrier genetic elements and environmental triggering factors. The establishment of international criteria for a diagnosis, clinical protocols were developed to aid the activity of the disease thus allowing the standardization of research in the area, although there are no laboratory or histopathological changes defined disease, the diagnosis dependent on a careful clinical evaluation that when are determinants for the prognosis. In this article we review clinical data for the diagnosis of Behçet's Disease.

Manifestações neurológicas da doença de Behçet / Neurological features of Behçet's disease

Introdução: A doença de Behçet (DB), também chamada síndrome de Behçet, é uma doença crônica, multissistêmica com remissões e exacerbações e evolução imprevisível. Objetivo: Descrever as manifestações neurológicas da doença de Behçet. Metodologia: Revisão narrativa da literatura utilizando o Pubmed como banco de dados. Resultados: Envolvimento neurológico em pacientes com DB conhecido como Neuro-Behcet (NB) apresenta uma prevalência variável de 2.2-5%. O diagnóstico de NB e baseado na presença de síndromes neurológicas em pacientes com achados sistêmicos de DB. Achados de neuroimagem e achados de líquor são uteis para excluir diagnósticos diferenciais, principalmente quadros infecciosos. Conclusão: Ainda que a DB seja rara na pratica neurológica rotineira, esta e comumente mencionada no diagnostico diferencial de doenças inflamatórias e/ou desmielinizantes do SNC.

Introductino: Behçet's disease (BD), also called Behçet's syndrome, is a chronic, multisystemic disease with remissions and exacerbations and unpredictable evolution. Objective: To describe neurological manifestations of BD. Metodology: Narrative review of the literature using Pubmed database. Results: Neurological involvement in patients with BD known as Neuro-Behcet (NB) has a variable prevalence of 2.2-5%. The diagnosis of NB is based on the presence of neurological syndromes in patients with systemic BD findings. Neuroimaging findings and cerebrospinal fluid findings are useful to exclude deferential diagnoses, especially infectious conditions. Conclusion: Although BD is rare in routine neurological practice, it is commonly mentioned in the differential diagnosis of inflammatory and / or demyelinating diseases of the CNS.

Optimizando el manejo de la uveítis con agentes biológicos en Chile del 2020 / Optimizing uveitis management with biological agents in Chile in 2020

Los agentes biológicos han irrumpido como una alternativa eficaz en el tratamiento de las uveítis no-infecciosas, especialmente en cuadros refractarios a inmunosupresores convencionales, con buena tolerancia y rápido efecto. Hay patologías como la enfermedad de Behçet en que incluso pueden estar indicados como tratamiento de primera línea. Este artículo ayudará a reconocer las patologías específicas donde presentan mayor eficacia, entrega herramientas para escoger el agente más adecuado para cada paciente y sugiere estrategias para evitar la pérdida de control de la enfermedad en el largo plazo.

Biological therapies have emerged as an effective option for the treatment of non-infectious uveitis, especially in refractive cases to conventional immunosup-pressive drugs. They are fast-acting, well tolerated, and can be considered as first-line agents for the treatment of certain uveitis like in Behçet ́s disease. This article will aid in identifying the uveitis syndromes where biological therapy is more effective, help choosing the most appropriate agent for a particular case and offer suggestions on how to keep long-term disease control.

Manifestaciones imagenológicas pulmonares y abdominales de las vasculitis primarias / Imaging of Pulmonary and Abdominal Manifestations of Primary Vasculitis

Las vasculitis son un grupo de enfermedades que consisten en la inflamación dentro y alrededor de la pared de los vasos sanguíneos, lo que produce necrosis, y su extensión depende del tamaño y ubicación del vaso afectado. Se pueden manifestar en cualquier órgano, por lo que se consideran enfermedades sistémicas. Las vasculitis primarias son patologías poco comunes y difíciles de diagnosticar, porque sus signos y síntomas son inespecíficos y pueden confundirse con muchas otras patologías ­como enfermedades infecciosas, neoplásicas o del tejido conjuntivo­. Por esta razón, para su diagnóstico es importante combinar los hallazgos clínicos, radiológicos, de estudios histopatológicos y de laboratorio. Esta revisión se enfocará en el papel de los hallazgos imagenológicos en el diagnóstico y monitoreo de las manifestaciones pulmonares y abdominales de las vasculitis más frecuentes.

Vasculitis are a group of diseases that are characterized by the presence of inflammation in and around the wall of the blood vessels. This produces necrosis and inflammation where the extension of the injury depends on the size and location of the affected vessel. They can have manifestations in any organ, so they are considered systemic diseases. Primary vasculitis are uncommon pathologies and difficult to diagnose because its signs and symptoms are nonspecific and are easily confused with many other disease processes such as infectious, neoplastic or connective tissue. Because of this, it is important to combine clinical, radiological, histopathological and laboratory findings to achieve a correct diagnosis. In this review, we will focus on the role of imaging findings on the diagnosis and monitoring for pulmonary and abdominal manifestations of the most frequent vasculitis.