SER recommendations on treatment of refractory Behçet's syndrome.

OBJECTIVE: To develop multidisciplinary recommendations based on available evidence and expert consensus for the therapeutic management of patients with refractory Behçet's syndrome (BS) (difficult to treat, severe resistant, severe relapse) to conventional treatment. METHODS: A group of experts identified clinical research questions relevant to the objective of the document. These questions were reformulated in PICO format (patient, intervention, comparison and outcome). Systematic reviews of the evidence were conducted, the quality of the evidence was evaluated following the methodology of the international working group Grading of Recommendations Assessment, Development, and Evaluation (GRADE). After that, the multidisciplinary panel formulated the specific recommendations. RESULTS: 4 PICO questions were selected regarding the efficacy and safety of systemic pharmacological treatments in patients with BS with clinical manifestations refractory to conventional therapy related to mucocutaneous and/or articular, vascular, neurological parenchymal and gastrointestinal phenotypes. A total of 7 recommendations were made, structured by question, based on the identified evidence and expert consensus. CONCLUSIONS: The treatment of most severe clinical manifestations of BS lacks solid scientific evidence and, besides, there are no specific recommendation documents for patients with refractory disease. With the aim of providing a response to this need, here we present the first official Recommendations of the Spanish Society of Rheumatology for the management of these patients. They are devised as a tool for assistance in clinical decision making, therapeutic homogenisation and to reduce variability in the care of these patients.

Recomendaciones SER sobre el tratamiento del síndrome de Behçet refractario / SER recommendations on treatment of refractory Behçet's syndrome

Objetivo: Elaborar recomendaciones multidisciplinares, basadas en la evidencia disponible y el consenso de expertos, para el manejo terapéutico de los pacientes con síndrome de Behçet refractario (difícil de tratar, resistente grave, recidivante grave) al tratamiento convencional. Métodos: Un panel de expertos identificó preguntas clínicas de investigación relevantes para el objetivo del documento. Estas preguntas fueron reformuladas en formato PICO –paciente, intervención, comparación, outcome o desenlace–. A continuación, se realizaron revisiones sistemáticas; la evaluación de la calidad de la evidencia se realizó siguiendo la metodología del grupo internacional de trabajo Grading of Recommendations, Assessment, Development, and Evaluation. Tras esto, el panel multidisciplinar formuló las recomendaciones. Resultados: Se seleccionaron 4 preguntas PICO relativas a la eficacia y seguridad de los tratamientos farmacológicos sistémicos en los pacientes con síndrome de Behçet con manifestaciones clínicas refractarias a terapia convencional, relacionadas con los fenotipos mucocutáneo y/o articular, vascular, neurológico-parenquimatoso y gastrointestinal. Se formularon un total de 7 recomendaciones estructuradas por pregunta, con base en la evidencia encontrada y el consenso de expertos. Conclusiones: El tratamiento de las manifestaciones clínicas más graves del síndrome de Behçet carece de evidencia científica sólida y no existen documentos de recomendaciones específicas para los pacientes con enfermedad refractaria a la terapia convencional. Con el fin de aportar una respuesta a esta necesidad, se presenta el primer documento de recomendaciones de la Sociedad Española de Reumatología específicas para el abordaje terapéutico de estos pacientes, que servirá de ayuda en la toma de decisiones clínica y la reducción de la variabilidad en la atención.(AU)

Objective: To develop multidisciplinary recommendations based on available evidence and expert consensus for the therapeutic management of patients with refractory Behçet's syndrome (difficult to treat, severe resistant, severe relapse) to conventional treatment. Methods: A group of experts identified clinical research questions relevant to the objective of the document. These questions were reformulated in PICO format –patient, intervention, comparison and outcome–. Systematic reviews of the evidence were conducted; the quality of the evidence was evaluated following the methodology of the international working group Grading of Recommendations, Assessment, Development, and Evaluation. After that, the multidisciplinary panel formulated the specific recommendations. Results: Four PICO questions were selected regarding the efficacy and safety of systemic pharmacological treatments in patients with Behçet's syndrome with clinical manifestations refractory to conventional therapy related to mucocutaneous and/or articular, vascular, neurological parenchymal and gastrointestinal phenotypes. A total of 7 recommendations were made, structured by question, based on the identified evidence and expert consensus. Conclusions: The treatment of most severe clinical manifestations of Behçet's syndrome lacks solid scientific evidence and, besides, there are no specific recommendation documents for patients with refractory disease. With the aim of providing a response to this need, here we present the first official recommendations of the Spanish Society of Rheumatology for the management of these patients. They are devised as a tool for assistance in clinical decision making, therapeutic homogenisation and to reduce variability in the care of these patients.(AU)

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.

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.

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)

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.

Neurological manifestations of Behçet's disease: Study of 57 patients. / Manifestaciones neurológicas en la enfermedad de Behçet: estudio de 57 pacientes.

INTRODUCTION: Prevalence of neurological involvement in Behçet's disease (BD) varies between studies and is associated with high morbimortality. Neurological involvement is classified as parenchymal and non-parenchymal involvement (vascular involvement). Treatment of neurological involvement consists of immunosuppressants. Evidence regarding presentation and outcomes in neuro-Behçet's (NB) is scarce. METHODS: Observational retrospective study that included consecutive patients diagnosed with BD between 2006 and 2019 in a tertiary hospital of Madrid. RESULTS: Fifty-seven patients were included with a mean follow-up of 7.83 (±6.98) years. The mean age at diagnosis of BD and first NB episode was 34.7 (± 12.1) and 31.03 (±9.9) years, respectively. Fifty-six NB episodes were registered in 25 patients. Fever (OR 3.39, CI 95% 1.14-10.15) and pseudofolliculitis (OR 4.06, CI 95% 1.11-14.80) were associated to NB. Trigeminal neuralgia was found in 16% (4/25) of patients. CONCLUSION: The prevalence of NB in patients with BD was higher than previously described. Fever and pseudofolliculitis were more frequently associated with NB, with low mortality during follow-up.

Methylation status of interleukin-6 gene promoter in patients with Behçet's disease.

BACKGROUND: IL-6 mRNA expression is significantly high in many autoimmune diseases such as Behçet's disease; this is often related with more aggressive phenotypes. Nevertheless, the essential molecular process for its high expression has not been completely realized. The aim of this study was undertaken to estimate the gene copy number variation and promoter methylation to IL-6's high expression. METHODS: This study was performed on 51 patients and 61 healthy controls. Initially, DNA and RNA were extracted from all specimens. Promoter methylation levels of IL-6 were evaluated by MeDIP-qPCR technique. Also, IL-6 gene expression was measured by Real-time PCR. After that, we evaluated the relationship between gene expression and methylation, as well as their relationship with clinical specification. RESULTS: As we expected, the expression level of IL-6 gene increased significantly in the patient group compared to the healthy subjects. Also, the relative promoter methylation level of the IL-6 mRNA was significantly lower in patient group compared to healthy group (p<0.001). DISCUSSION: We disclosed that the promoter hypomethylation may be considered as one of the main defects for IL-6 mRNA high expression in patients with Behçet's disease.

Síndrome de Behçet complicado con trombosis venosas profundas en miembros inferiores / Behçet syndrome complicated with deep vein thrombosis in lower limbs

En este trabajo se presentó a un paciente de 32 años que ingresó por pérdida de peso, decaimiento, orinas turbias y dolor en región inguinal derecha. Se describió la evolución clínica, los estudios imagenológicos y de laboratorio que permitieron diagnosticar la presencia del Síndrome de Behçet. Los principales signos presentados por el paciente fueron: ulceraciones bucales recurrentes, la presencia de uveítis anterior y trombosis venosas profundas en miembros inferiores. El cuadro clínico del paciente y la valoración conjunta de varias especialidades médicas constituyeron los factores claves para establecer el diagnóstico definitivo. La evolución del paciente fue satisfactoria con el tratamiento de prednisona y anticoagulantes(AU)

This paper reports a 32-year-old male patient who was hospitalized for weight loss, decay, cloudy urine and pain in the right inguinal region. The clinical evolution, imaging and laboratory studies allowed diagnosing Behçet Syndrome. The patient showed main signs as recurrent mouth ulcerations, anterior uveitis and deep vein thrombosis in the lower limbs. The clinical condition of this patient and the joint assessment of various medical specialties were the key factors in establishing the definitive diagnosis. The patient´s evolution was satisfactory treated with prednisone and anticoagulants(AU)

Relationship between erythrocyte sedimentation rate and choroidal and retinal thickness in Behçet's disease / A relação entre a taxa de sedimentação eritrocitária, espessuras de coroide e retina na doença de Behçet

ABSTRACT Purpose: To compare retinal and choroidal thickness in patients with Behçet's disease with and without ocular involvement as well as to evaluate the correlation between erythrocyte sedimentation rate and choroidal thickness among patients with Behçet's disease. Methods: This was a prospective interventional study investigating erythrocyte sedimentation as well as choroidal and retinal thickness among patients with Behçet's disease. Patients who were diagnosed based on The International Criteria for Behçet's Disease with (Group A) or without (Group B) ocular involvement and a matched control group (Group C) participated in the study. Optical coherence tomography measurements and blood tests were performed on the same day. Retinal and choroidal thickness were measured using spectral-domain optical coherence tomography (Spectralis, Heidelberg Engineering, Heidelberg, Germany), and central macular thickness, central subfoveal choroidal thickness, and retinal nerve fiber layer thickness were measured using optical coherence tomography. Results: Average erythrocyte sedimenta­tion values were 9.89 mm/h in Group A, 16.21 mm/h in Group B, and 3.89 mm/h in Group C; average central subfoveal choroidal thickness values were 350.66, 331.74, and 325.95 mm, respectively. Average central macular thickness and retinal nerve fiber layer thickness values of patients in Groups A, B and C were 226.39 and 225.97mm; 234.11 and 92.00 mm; and 97.58 and 99.84 mm, respectively. No significant difference was seen between Group A and B patients in central subfoveal choroidal thickness, central macular thickness, or retinal nerve fiber layer thickness values. Central macular thickness was statistically significantly thinner in Groups A and B than in Group C (p=0.016). Group A had thinning in the nasal quadrant of the retinal and general retinal nerve fiber layers when compared with those in Group C (p=0.010 and 0.041, respectively). A connection could not be established between the erythrocyte sedimentation, central subfoveal cho­roidal thickness, central macular thickness, and retinal nerve fiber layer thickness in the patients with Behçet's disease. Conclusion: The erythrocyte sedimentation rate is typically used to test for activation of Behçet's disease and assess treatment response. In our study, we could not establish a connection between the erythrocyte sedimentation rate and central subfoveal choroidal thickness, central macular thickness, and retinal nerve fiber layer thickness in patients with systematically active Behçet's disease without ocular involvement.

RESUMO Objetivos: Comparar a espessura da retina e da coroide em pacientes com doença de Behçet, com e sem acometimento ocular e avaliar a correlação entre a taxa de sedimentação de eritrócitos e a espessura da coroide em pacientes com doença de Behçet. Métodos: Estudo prospectivo intervencional que investigou a sedimentação de eritrócitos, espessura de coroide e da retina em pacientes com doença de Behçet. Os pacientes que foram diagnosticados com base nos Critérios Internacionais para a Doença de Behçet com (Grupo A) ou sem (Grupo B) envolvimento ocular e um grupo controle correspondente (Grupo C) participaram do estudo. Medidas de tomografia de coerência óptica e exames de sangue foram realizados no mesmo dia. As espessuras da retina e da coroide foram medidas utilizando tomografia de coerência óptica de domínio espectral (Spectralis, Heidelberg Engineering, Hidelberg, Germany) e a espessura macular central, a espessura coroidal subfoveal central e a espessura da camada de fibra nervosa da retina foram medidas usando tomografia de coerência óptica. Resultados: Os valores médios de sedimentação de eritrócitos foram de 9,89 mm/h no Grupo A, 16,21 mm/h no Grupo B e 3,89 mm/h no Grupo C; os valores médios da espessura da coroide subfoveal central foram 350,66, 331,74 e 325,95 mm respectivamente. Os valores médios da espessura macular central e da espessura da camada de fibra nervosa da retina dos pacientes nos grupos A, B e C foram de 226,39, 225,97, 234,11 mm e 92,00, 97,58, 99,84 mm respectivamente. Não houve diferença significativa entre pacientes do Grupo A e B na espessura da coroide subfoveal central, espessura macular central ou valores da espessura da camada de fibra nervosa da retina. A espessura macular central foi estatisticamente significativamente mais fina nos Grupos A e B do que no Grupo C (p=0,016). O Grupo A apresentou afinamento na porção nasal das camadas retiniana e geral da fibra nervosa da retina quando comparado com o Grupo C (p=0,010, p=0,041, respectivamente). Não foi possível estabelecer uma conexão entre a sedimentação dos eritrócitos, a espessura subfoveal central da coroide, a espessura macular central e espessura da camada de fibras nervosas da retina nos pacientes com doença de Behçet. Conclusão: A taxa de sedimentação de eritrócitos é comumente utilizada para testar a ativação da doença de Behçet e avaliar a resposta ao tratamento. Em nosso estudo, não foi possível estabelecer uma conexão entre a taxa de sedimentação de eritrócitos e a espessura da coroide subfoveal central, espessura macular central e espessura da camada de fibras nervosas da retina em pacientes com doença de Behçet sistematicamente ativa sem envolvimento ocular.

Tratamento endovascular da doença de Behçet: relato de caso / Endovascular treatment for Behçet's disease: a case report

A doença de Behçet (DB) é uma vasculite multissistêmica de etiologia desconhecida. O acometimento cardiovascular é bem descrito na literatura, sendo que a principal causa de morte na DB é secundária a complicações aneurismáticas. Relata-se aqui o caso de um paciente portador de DB com aneurisma de aorta abdominal reincidente, o qual foi corrigido por meio de endoprótese customizada. O tratamento ideal para portadores de DB ainda é bastante controverso e desafiador, tendo em vista as dificuldades técnicas e as recidivas frequentes. A intervenção endovascular apresenta-se como uma alternativa viável e consideravelmente menos mórbida do que a cirurgia convencional

Behçet's disease (BD) is a multisystemic vasculitis of unknown etiology. Cardiovascular involvement has been thoroughly described in the literature and the major cause of death in BD is secondary to aneurysm complications. In this case report, a patient with BD presented with a recurrent abdominal aortic aneurysm, which was corrected using a custom-made endoprosthesis. The optimal treatment for patients with BD remains highly controversial and challenging because of technical difficulties and frequent recurrence. Endovascular intervention seems to be a feasible alternative with considerably less morbidity than conventional surgery

Neurobehcet, a propósito de un caso / Neurobechet, about a case

El neurobehcet (NB) es una manifestación poco frecuente y grave de la enfer-medad de Behcet (EB) con diferentes formas de presentación según el aérea del sistema nervioso que se vea afectada. Se presenta el caso de una paciente de sexo femenino de 38 años de edad con historia de larga data de psicosis de posible cau-sa orgánica. Durante su última hospitalización por deterioro funcional y cognitivo se rescata el antecedente de úlceras orales y genitales y un episodio previo de es-cleritis. Se complementa el estudio con punción lumbar, resonancia y angioreso-nancia nuclear magnética de cerebro y antígeno de histocompatibilidad los cuales sugieren el diagnóstico de NB. Se trata con corticoides e inmunosupresores, pero a pesar de mejoría en el líquido cefalorraquídeo no hay mejoría clínica, que se presume sería consecuencia de lo tardío del diagnóstico y tratamiento.

Neurobehcet (NB) is a rare and serious manifestation of Behcet's disease (EB), with different forms of presentation depending on the area of the nervous system involved. A clinical case of a 38-year-old female patient with a long-standing his-tory of psychosis of possible organic cause is presented. During her last hospital-ization due to functional and cognitive impairment the history of oral and genital ulcers and a previous episode of scleritis is obtained. The study is complemented with a lumbar puncture, magnetic resonance, magnetic nuclear angioresonance of the brain and histocompatibility antigen which suggests the diagnosis of NB. It is treated with corticosteroids and immunosuppressants, but despite improve-ment in cerebrospinal fluid there is no clinical improvement, which is presumed to be a consequence of the late diagnosis and treatment.

Manifestações bucais e considerações gerais da síndrome de Behçet: relato de caso / Oral manifestations and general considerations of Behçet's Syndrome: case report

Objetivo: relatar um caso de síndrome de Behçet, enfatizando o controle das manifestações bucais. Relato de caso: paciente de 21 anos de idade, já diagnosticado com síndrome de Behçet há 7 anos, sem histórico familiar, relatou o aparecimento de úlceras recorrentes, quando iniciou tratamento ortodôntico. Procurou a clínica odontológica da Universidade Estadual de Maringá queixando-se de "aftas e carne crescida" na boca. Foi realizada biópsia excisional do nódulo, e o exame histopatológico revelou tecido hiperplásico com infiltrado inflamatório crônico decorrente. Após 45 dias, observaram-se presença de novas aftas em mucosa labial, língua e gengiva assim como total cicatrização na região da biópsia. Atualmente, o paciente permanece em controle clínico médico e odontológico, estando as lesões aftosas presentes em diferentes regiões. Considerações finais: apesar de rara, essa doença é de grande relevância para o cirurgião-dentista, que deve saber diagnosticá-la e controlar suas manifestações bucais. Além disso, o acompanhamento é necessário para melhorar a qualidade de vida do paciente. (AU)

Objective: report a case of Behçet's Syndrome, emphasizing the control of oral manifestations. Case report: a 21-year-old patient, diagnosed with Behçet's Syndrome for 7 years, with no family history, reporting recurrent ulcers when orthodontic treatment was started. He sought a dental clinic at the State University of Maringá complaining of "aphthae and grown flesh" in the mouth. Excisional nodule biopsy and histopathological examination of hyperplastic tissue with chronic inflammatory infiltrate were performed. After 45 days, the presence of new aphthae were observed in the labial mucosa, tongue and gingiva, as well as total scarring in the region of the biopsy. Currently, the patient remains in clinical and dental control, remaining as aphthous interventions present in different regions. Final considerations: although rare, this disorder is of great relevance for the dental practioner, who must know how to diagnose and control its oral manifestations. In addition, follow-up is necessary to improve the patient's quality of life. (AU)

Doença de Behçet ocular: a nossa realidade / Behçet disease: our experience

Resumo Objetivo: A doença de Behçet é uma vasculite inflamatória sistémica, de etiologia desconhecida. Pode atingir virtualmente todos os sistemas, sendo as manifestações oculares comuns. O objectivo deste trabalho foi analisar doentes com doença de Behçet ocular quanto aos parâmetros demográficos, manifestações clinicas, terapêutica e principais complicações. Métodos: Estudo descritivo e retrospetivo, que incluiu 11 doentes com o diagnóstico de doença de Behçet ocular, segundo os critérios do ISG, observados na consulta de Inflamação Ocular do Hospital Prof. Doutor Fernando da Fonseca nos últimos 3 anos. Resultados: Identificaram-se 11 doentes, 5 homens e 6 mulheres, todos caucasianos. A idade média ao diagnóstico foi de 33,45±6,49 anos. A manifestação ocular foi o primeiro sinal da doença em 2 doentes. Em 72,7% dos casos as manifestações oculares foram bilaterais. Identificaram-se 4 casos de panuveíte, 3 de uveíte posterior, 2 de uveíte anterior, 1 de queratite e 1 caso de episclerite. O glaucoma e a catarata foram a complicação ocular mais frequente. O tratamento sistémico incluiu a corticoterapia oral em associação com terapêutica adjuvante imunossupressora, sendo os mais utilizados a azatioprina e a ciclosporina. Em 3 doentes houve necessidade de terapêutica biológica com infliximab para controlo da doença. Conclusão: A manifestação ocular mais frequente foi a panuveíte. Esta doença pode condicionar complicações oculares com diminuição irreversível da acuidade visual. A orientação destes doentes exige uma abordagem global e interdisciplinar.

Abstract Objective: Behçet's disease is a systemic inflammatory vasculitis of unknown etiology. It can virtually reach all systems, being common ocular manifestations. The aim of this study was to analyze patients with ocular Behcet's disease regarding demographic parameters, clinical manifestations, therapeutic approach and main complications. Methods: Descriptive and retrospective study, including 11 patients diagnosed with ocular Behcet's disease, according to the ISG criteria, observed in the Ocular Inflammation appointment of the Hospital Prof. Doutor Fernando da Fonseca in the last 3 years. Results: We identified 11 patients, 5 males and 6 females, all caucasian. The mean age at diagnosis was 33.45 ± 6.49 years. Ocular manifestation was the first sign of the disease in 2 patients. In 72.7% of the cases, ocular manifestations were bilateral. There were four cases of panuveitis, 3 of posterior uveitis, 2 of anterior uveitis, 1 of keratitis and 1 case of episcleritis. Glaucoma and cataract were the most frequent ocular complications. Systemic treatment included oral corticosteroid therapy in combination with adjuvant immunosuppressive therapy, and the most commonly used was azathioprine and cyclosporine. In 3 patients there was a need for biological treatment with infliximab to control the disease. Conclusion: The most frequent ocular manifestation was panuveitis. This disease can promote eye complications with an irreversible decrease in visual acuity. Orientation of these patients requires a global and interdisciplinary approach.

Enfermedad de Behçet y embarazo: a propósito de un caso / Behçet's disease and pregnancy: a propos of a case

Introducción: La enfermedad de Behçet es una enfermedad crónica, multisistémica, de causa desconocida, caracterizada clínicamente por la presencia de aftas orales, genitales, manifestaciones sistémicas y prueba de patergia positiva. Es una enfermedad poco frecuente y predomina en el sexo masculino, por lo que su relación con el embarazo es escasamente reportada en la literatura. Objetivo: Mostrar las manifestaciones clínicas, evolución y complicaciones presentes durante el embarazo en una paciente con enfermedad de Behçet. Presentación del Caso: Se presenta el caso de una paciente de 26 años de edad con diagnóstico de Enfermedad de Behçet, la cual presentó complicaciones durante su embarazo que tuvieron como resultado un producto final de la gestación bajo peso. Conclusiones: La Enfermedad de Behçet puede provocar complicaciones sobre el embarazo como las presentadas en este caso, a su vez el embarazo puede producir actividad de la enfermedad, por lo que lograr un adecuado control de esta previo al embarazo y llegar a tener una correcta planificación familiar son vitales para llegar a un feliz término del embarazo en estas pacientes(AU)

Introduction: Behçet's disease is a chronic and multisystem illness, of unknown cause, clinically characterized by the presence of oral sores, genital, systemic manifestations and positive pathergy test. It is a not very frequent illness and prevails in the masculine sex; due to this its relationship with the pregnancy is barely reported in the literature. Objective: To show the clinical manifestations, evolution and complications that are present during the pregnancy in a patient with Behçets illness. Cases presentation: A case of a 26-year-old patient with diagnose of Behçes illness is presented, which show complications during its pregnancy that result at the end of her pregnancy; and underweight. Conclusions: The Behçets illness could cause complications during pregnancy like those presented in this case, in turn the pregnancy can produce activity of the illness, and for that reason, to achieve an appropriate control of the illness before the pregnancy with the aim of having a correct family planning, which is vital to arrive to a happy end of the pregnancy in these patients(AU)

Rupture of celiac trunk aneurysm in patient with Behçet Disease / Ruptura de aneurisma de tronco celíaco em paciente com Doença de Behçet

ABSTRACT We report a case of a ruptured aneurysm of the celiac trunk in a 32-year-old, male patient with Behçet Disease (BD). Aneurysm resection was performed and the patient is well during a follow up of 32 months. To our knowledge, this is the first reported case of a ruptured celiac trunk aneurysm successfully treated in a patient with BD.

RESUMO Relatamos o caso de um aneurisma roto do tronco celíaco em um paciente de 32 anos, do sexo masculino, portador de Doença de Behçet (DB). A ressecção do aneurisma foi realizada e o paciente está bem, com acompanhamento de 32 meses. Até onde sabemos, este é o primeiro caso relatado de um aneurisma do tronco celíaco roto tratado com sucesso em um paciente com DB.