Analysis of the clinical characteristics of 25 cases with parenchymal neuro-Behcet's disease / 中华内科杂志

Objective: To retrospectively investigate the clinical data, radiological characteristics, treatment, and outcome of patients with parenchymal neuro-Behcet's disease (P-NBD) with particular emphasis on dizziness. Methods: This was a cross-sectional study of clinical data from 25 patients with a confirmed diagnosis of P-NBD who were admitted to the Department of Neurology of the First Medical Center of Chinese People's Liberation Army General Hospital between 2010 and 2022. The median age of the population was 37 years (range: 17-85 years). Clinical data were retrospectively analyzed, including gender, age of onset, disease duration, clinical manifestations, serum immune indicators, cerebrospinal fluid (CSF) routine biochemical and cytokine levels, cranial and spinal magnetic resonance imaging (MRI) findings, treatment, and outcome. Results: The majority of patients were male (16 cases; 64.0%), the mean age of onset was (28±14) (range: 4-58 years), and the disease course was acute or subacute. Fever was the most common clinical presentation, and the complaint of dizziness was not uncommon (8/25 patients). Analysis of serum immune indices, including complement (C3 and C4), erythrocyte sedimentation rate, interleukin-1 (IL-1), IL-6, IL-8 and tumor necrotic factor-alpha were abnormal in 80.0% of patients (20/25). Most of the 16/25 patients who underwent lumbar puncture tests had normal intracranial pressure and increased CSF white cell count and protein [median values were 44 (15-380) ×106/L and 0.73 (0.49-2.81) g/L, respectively]. Of the five patients who underwent CSF cytokine tests, four patients had abnormal results; of these, an elevated level of IL-6 was most common, followed by IL-1 and IL-8. The most common site of involvement in cranial MRI was the brainstem and basal ganglia (60.0% respectively), followed by white matter (48.0%) and the cortex (44.0%). Nine cases (36.0%) showed lesions with enhancement and six cases (24.0%) showed mass-like lesions. Three patients (12.0%) patients had lesions in the spinal cord, most frequently in the thoracic cord. All patients received immunological intervention therapy; during follow up, the majority had a favorable outcome. Conclusions: P-NBD is an autoimmune disease with multiple system involvement and diverse clinical manifestations. The symptom of dizziness is not uncommon and can be easily ignored. Early treatment with immunotherapy is important and can improve the outcome of these patients.

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.

Behçet’s disease presenting with recurrent vulvar abscesses, oral ulcers, and skin pustules in the Philippines

@#Behçet's disease (BD) is a systemic vasculitis characterized by recurrent acute vascular inflammation. There is currently limited epidemiological data in Southeast Asia. We discuss a 26-year-old woman who came to our institution for recurrent vulvar abscesses for a year. Physical examination showed labial, tongue, and vulvar ulcers. Pathergy test was negative and biopsy done were unremarkable . We diagnosed her clinically as a case of Behçet’s disease. We started her on colchicine 0.6 mg once daily and educated her on skin, oral, and perineal hygiene. On follow-up after one month, she noted resolution of her lesions. However, symptoms recurred in the last three months after discontinuing treatment. We used telemedicine to communicate due to the COVID-19 pandemic and lockdown, advising her to continue her medication. Timely diagnosis is essential in order to provide symptomatic relief and enhance patient education.

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.

Association of complementary and integrative therapy use and symptoms among Turkish patients with familial Mediterranean fever / 中西医结合学报

OBJECTIVE@#This study explored the correlations between the use of complementary and integrative therapies (CITs) and symptoms among Turkish patients with familial Mediterranean fever (FMF).@*METHODS@#This is a cross-sectional and descriptive study. The study was conducted with 1119 FMF patients who were registered to the social networking site for Behcet's and the FMF Patients Association (Befemder) in Turkey, between January 2018 and February 2019. Data were collected using an online survey, for which a three-part questionnaire was created using a Google form. Descriptive statistics, chi-square test and logistic regression analysis were used to analyze the data.@*RESULTS@#It was determined that 53.2% of the individuals who participated in the research used various forms of CITs and that 32.8% used vitamin and mineral supplements (calcium, iron, and vitamin B12, C and D), 25.0% used nutritional supplements (fish oil and honey), and 24.6% used oral herbs (ginger, turmeric, green tea and rosemary) and mind-body methods (relaxation, respiration exercise and meditation). It was determined that the percentage of participants that used CITs was higher among women (odds ratio [OR] = 1.825; 95% confidence interval [CI] 1.421-2.344), those with joint pain (OR = 1.385; 95% CI 1.047-1.832), those with difficulty breathing (OR = 1.323; 95% CI 1.031-1.697), those with gastrointestinal symptoms (OR = 1.405; 95% CI 1.089-1.814) and those who had a family member with FMF (OR = 1.437; 95% CI 1.115-1.851).@*CONCLUSION@#More than half of the individuals used at least one type of CIT for symptom control.

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.