RESUMO
BACKGROUND: Behçet's disease (BD) is a chronic vasculitic multi-systemic disease of unknown etiology. BD is characterized by recurrent attacks of oral aphthae, genital ulcers, and uveitis. BD is a multisystemic disorder and as such it may provoke various psychiatric manifestations, including depression. OBJECTIVES: To evaluate the association between BD and depression, adjusting for established risk factors for depression. METHODS: We executed a cross-sectional study based on the Clalit Health Services database, the largest healthcare organization in Israel, serving over 4.4 million members. For this study 873 BD patients were detected and matched with 4369 controls by age and sex. RESULTS: The rate of depression was higher among the BD patients compared with the control group (9.39% vs 5.49%, respectively, odds ratio [OR] 1.79, 95% confidence interval [95%CI] 1.37-2.31, P < 0.001). An association between BD and depression was also observed on multivariable analysis (OR 1.83, 95%CI 1.39-2.39, P < 0.001). When stratifying the data, according to established risk factors, the association between BD and depression was prominent in the youngest age group (18-39 years of age), low and high socioeconomical status, and non-smokers. CONCLUSIONS: Establishing the association between BD and depression should influence the attitude and the treatment of BD patients, as this relationship requires a more holistic approach and a multidisciplinary treatment regimen for all patient needs.
Assuntos
Síndrome de Behçet , Estomatite Aftosa , Uveíte , Humanos , Adolescente , Adulto Jovem , Adulto , Síndrome de Behçet/complicações , Síndrome de Behçet/epidemiologia , Estudos Transversais , Depressão/epidemiologia , Depressão/etiologiaRESUMO
Corticosteroid-induced central serous chorioretinopathy (CSCR) has been reported to develop in many intraocular inflammatory diseases and usually resolves spontaneously after discontinuation of corticosteroids. Patients without any improvement may require alternative therapies. In this case report, we present the case of a 35-year-old man with Behçet's disease who had complaints of decreased vision due to CSCR in his left eye while using systemic corticosteroids along with cyclosporine and azathioprine. Half-fluence photodynamic therapy (PDT) was performed because the CSCR did not regress despite discontinuation of systemic corticosteroids. After treatment, his visual acuity increased with complete resolution of the subfoveal fluid. Half-fluence PDT seems to be an effective and safe treatment for patients who develop acute CSCR while under systemic or local corticosteroid therapy for intraocular inflammatory diseases such as Behçet's uveitis and do not improve despite steroid discontinuation.
Assuntos
Síndrome de Behçet , Coriorretinopatia Serosa Central , Fotoquimioterapia , Uveíte , Corticosteroides/uso terapêutico , Adulto , Síndrome de Behçet/complicações , Síndrome de Behçet/diagnóstico , Síndrome de Behçet/tratamento farmacológico , Coriorretinopatia Serosa Central/induzido quimicamente , Coriorretinopatia Serosa Central/diagnóstico , Coriorretinopatia Serosa Central/tratamento farmacológico , Angiofluoresceinografia , Humanos , Masculino , Fotoquimioterapia/efeitos adversos , Fármacos Fotossensibilizantes/efeitos adversos , Tomografia de Coerência Óptica , Uveíte/tratamento farmacológico , Verteporfina/uso terapêuticoRESUMO
Atypical Behçet's is recognised in myelodysplastic syndrome (MDS) cases and is associated with trisomy 8. Clonal cytopenia of undetermined significance (CCUS) is recognised as a precursor to MDS. Our case describes the presentation of atypical Behçet's, in association with CCUS, post a Streptococcal infection. A mutation of a zinc finger RNA spliceosome, ZRSR2, is also described. Our patient initially presented with macrocytic anaemia, together with neutropenia and lymphocytopenia on routine monitoring. Later gastrointestinal symptoms together with oral and anal ulcerations developed. He was treated with oral zinc therapy and had resolution of recurrent oral ulcerations and significant reduction in severity of anal ulcerations. The functional impact of ZRSR2 mutation on spliceosome assembly is yet to be defined, but has been previously reported in CCUS with a clinical phenotype of macrocytic anaemia.
Assuntos
Anemia Macrocítica , Síndrome de Behçet , Síndromes Mielodisplásicas , Anemia Macrocítica/diagnóstico , Anemia Macrocítica/tratamento farmacológico , Anemia Macrocítica/genética , Síndrome de Behçet/complicações , Síndrome de Behçet/diagnóstico , Síndrome de Behçet/tratamento farmacológico , Hematopoiese Clonal , Humanos , Masculino , Síndromes Mielodisplásicas/complicações , Síndromes Mielodisplásicas/diagnóstico , Síndromes Mielodisplásicas/tratamento farmacológico , Zinco/uso terapêuticoRESUMO
BACKGROUND: Cutaneous ulcers of Behçet disease (BD) are rare but have high morbidity and resistance to conventional therapies. An important and essential aspect of ulcer management is debridement. Regarding maggot therapy (MT), excretions of the green bottle fly, Lucilia sericata, have been shown to have the ability to remove necrotic debris and promote healing. AIM: To evaluate the efficacy of MT for cutaneous ulcers of BD. METHODS: In this open-label trial, patients with BD with refractory leg ulcers suitable for MT were enrolled. Maggot application was performed until complete debridement was achieved, and all patients were followed up for 12 months afterwards to assess the total healing of ulcers. RESULTS: In total, 24 patients with 32 ulcers were enrolled. Using MT, 91.6% of all ulcers were completely debrided. Mean time to debridement was 14.9 days and mean number of cycles required was 5.3. Mean ulcer size was decreased by 23% with treatment. Time to debridement was positively correlated with pretreatment ulcer size and ulcer duration (P = 0.01 and P < 0.01) but not with ulcer depth, comorbidities, smoking, age or sex (P > 0.05 for all). During follow-up, 79.1% of all ulcers healed completely. Mean time required for total healing was positively correlated with ulcer duration, pretreatment and post-treatment ulcer area, ulcer depth and mean time to total debridement (P < 0.03, P = 0.00, P = 0.04 and P < 0.01, respectively). CONCLUSIONS: To our knowledge, the findings presented in this first and unique study may provide key answers about factors affecting success rate of MT in BD cutaneous ulcers.
Assuntos
Síndrome de Behçet/complicações , Desbridamento/métodos , Úlcera da Perna/etiologia , Úlcera da Perna/cirurgia , Adulto , Animais , Desbridamento/efeitos adversos , Desbridamento/estatística & dados numéricos , Procedimentos Cirúrgicos Dermatológicos/tendências , Dípteros/enzimologia , Dípteros/fisiologia , Feminino , Seguimentos , Humanos , Larva/enzimologia , Larva/fisiologia , Úlcera da Perna/patologia , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Resultado do Tratamento , Turquia/epidemiologia , Cicatrização/fisiologiaRESUMO
BACKGROUND To relieve the signs and symptoms of anterior uveitis (AU), an ocular manifestation of Behcet's syndrome, we prescribed a synbiotic supplementation (probiotics and prebiotics) for a 49-year-old woman. CASE REPORT Seven strains of bacteria - Lactobacillus casei, Lactobacillus rhamnosus, Streptococcus thermophilus, Bifidobacterium breve, Lactobacillus acidophilus, Bifidobacterium longum, and Lactobacillus bulgaricus, each 108 colony-forming units (CFU) - and fructo-oligosaccharide (FOS; 100 mg) were given as a capsule 2 times per day. After 7-month treatment, AU was improved and serum inflammatory markers - C-reactive protein (CRP), high-sensitivity CRP (hs-CRP), and estimated sedimentation rate (ESR) - were suppressed. Now, if a mild AU attack occurs, the problem is resolved by treatment with 1 gtt (from the Latin "guttae", meaning drops) eye drop (prednisolone 1%) for 1 week. CONCLUSIONS Synbiotic supplementation may contribute to treating AU, which is one of the most disastrous manifestations of BS, by controlling the proinflammatory processes.
Assuntos
Síndrome de Behçet/complicações , Suplementos Nutricionais , Probióticos/administração & dosagem , Simbióticos/administração & dosagem , Uveíte Anterior/tratamento farmacológico , Uveíte Anterior/etiologia , Síndrome de Behçet/diagnóstico , Feminino , Humanos , Lactobacillus acidophilus , Lacticaseibacillus casei , Lacticaseibacillus rhamnosus , Pessoa de Meia-Idade , Prognóstico , Recidiva , Medição de Risco , Índice de Gravidade de Doença , Resultado do Tratamento , Uveíte Anterior/fisiopatologiaRESUMO
BACKGROUND/PURPOSE: Behcet's disease (BD) patients may have one or more hematinic deficiencies. This study evaluated whether there were significantly higher frequencies of hemoglobin, iron, vitamin B12, and folic acid deficiencies and of hyperhomocysteinemia in BD patients. METHODS: The blood hemoglobin, iron, vitamin B12, folic acid, and homocysteine concentrations in 63 BD patients were measured and compared with the corresponding levels in 126 age- and sex-matched healthy control subjects. RESULTS: We found that 30.2%, 34.9%, 6.3%, 6.3%, and 14.3% of 63 BD patients had hemoglobin, iron, vitamin B12, and folic acid deficiencies and hyperhomocysteinemia, respectively. Both 63 BD and 19 major-typed RAS/BD patients had significantly higher frequencies of hemoglobin, iron, vitamin B12, and folic acid deficiencies and of hyperhomocysteinemia than 126 healthy control subjects (all P-values < 0.05). Moreover, 44 minor-typed RAS/BD patients had significantly higher frequencies of hemoglobin and iron deficiencies than 126 healthy control subjects (both P-values < 0.001). Of the 19 anemic BD patients, one had pernicious anemia, one had macrocytic anemia, 12 had normocytic anemia, four had iron deficiency anemia, and one had thalassemia trait-induced anemia. CONCLUSION: There are significantly higher frequencies of hemoglobin, iron, vitamin B12, and folic acid deficiencies and of hyperhomocysteinemia in BD patients. The normocytic anemia is the most common type of anemia in our 63 BD patients. We suggest that supplementations of vitamin BC capsules plus deficient vitamin B12 and/or folic acid may reduce the abnormally high serum homocysteine level to a relatively lower level in BD patients with hyperhomocysteinemia.
Assuntos
Anemia/sangue , Síndrome de Behçet/sangue , Deficiência de Ácido Fólico/sangue , Hiper-Homocisteinemia/sangue , Deficiência de Vitamina B 12/sangue , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Síndrome de Behçet/complicações , Estudos de Casos e Controles , Índices de Eritrócitos , Feminino , Ácido Fólico/sangue , Hematínicos , Hemoglobinas/análise , Homocisteína/sangue , Humanos , Ferro/sangue , Masculino , Pessoa de Meia-Idade , Taiwan , Vitamina B 12/sangue , Adulto JovemRESUMO
Onset of neurological symptoms early after intranodal lymphangiography can occur due to Lipiodol droplet migration through intrapulmonary lymphovenous communication. Patients with Behçet's disease may be at higher risk of developing this devastating complication.
Assuntos
Síndrome de Behçet/complicações , Quilotórax/terapia , Meios de Contraste/efeitos adversos , Embolização Terapêutica/efeitos adversos , Óleo Etiodado/efeitos adversos , Embolia Intracraniana/induzido quimicamente , Linfografia/efeitos adversos , Adulto , Síndrome de Behçet/diagnóstico , Quilotórax/complicações , Quilotórax/diagnóstico por imagem , Meios de Contraste/administração & dosagem , Imagem de Difusão por Ressonância Magnética , Óleo Etiodado/administração & dosagem , Evolução Fatal , Feminino , Humanos , Embolia Intracraniana/diagnóstico por imagem , Embolia Intracraniana/terapia , Tomografia Computadorizada por Raios XRESUMO
OBJECTIVES: To determine the types and to assess the role of auditory evoked potentials and otoacoustic emissions in early detection of hearing abnormalities in Behçet's disease (BD) patients. Their correlations with disease activity were also considered. METHODS: Thirty patients with BD and thirty apparently sex- and age-matched healthy volunteers were included in this study. Auditory evaluation included pure tone audiometry (PTA), otoacoustic emissions (TEOAEs, DPOAE), auditory brainstem response test (ABR) and cortical auditory evoked potentials (tone and speech CAEPs) for all patients and control. RESULTS: The highest abnormality of CAEP latencies elicited by (500Hz and 1000 Hz) as well as speech stimuli (da and ga) among our BD patients was delayed P1 and N1 waves at 80 dB with greater bilateral affection, as well as significant differences between patients and controls. All our BD patients had a smaller amplitude of distortion product OAE (DPOAE) and S/N ratio at 1, 2, 4, 6 kHZ compared with controls and the differences were highly statistically significant (p=0.0001). CONCLUSIONS: Being one of the autoimmune inner ear diseases (AIED), BD has a definite hearing impairment, even in the presence of normal hearing sensitivity, as evidenced by PTA. BD patients had a sub-clinical cochlear pathology which was not affected by disease activity or different organ affection. DPOAE (S/N ratio) proved to be a sensitive test in detecting minimal changes in cochlear pathology and the latencies of CAEPs (tone and speech) measures were considered as sensitive indicators (100%) of early detection of hearing impairment in BD patients.
Assuntos
Vias Auditivas/fisiopatologia , Síndrome de Behçet/complicações , Potenciais Evocados Auditivos do Tronco Encefálico , Transtornos da Audição/diagnóstico , Testes Auditivos/métodos , Emissões Otoacústicas Espontâneas , Estimulação Acústica , Adulto , Audiometria de Tons Puros , Audiometria da Fala , Limiar Auditivo , Síndrome de Behçet/diagnóstico , Estudos de Casos e Controles , Diagnóstico Precoce , Eletroencefalografia , Feminino , Transtornos da Audição/etiologia , Transtornos da Audição/fisiopatologia , Humanos , Masculino , Valor Preditivo dos Testes , Tempo de Reação , Índice de Gravidade de Doença , Adulto JovemRESUMO
BACKGROUND: Aortic rupture in Behcet's disease is associated with high mortality rates. Endovascular approach with parallel graft technique can be a valid solution in emergency setting for the treatment of thoraco-abdominal aorta involvement in Behcet's disease. METHODS: We describe the clinical case of a 41-year-old man, urgently hospitalized for a rupture of thoraco-abdominal aorta with occlusion of the celiac trunk and involvement of superior mesenteric artery. The patient also presented a massive right hemothorax and a massive left pulmonary thromboembolism. An aortic endoprosthesis was deployed from the third bottom of the thoracic aorta to the origin of the renal arteries. Parallel stent-graft technique was used to preserve the patency of the superior mesenteric artery. The procedure was performed with local anaesthesia due to the critical conditions of the patient. The patient was discharged in good clinical conditions. RESULTS: The angio-computed tomography performed 6 months after surgery showed good deployment of the endoprosthesis and patency of the superior mesenteric artery. CONCLUSION: In high risk for surgery patients, endovascular treatment with parallel stent graft of thoraco-abdominal aorta rupture is safe and feasible.
Assuntos
Aneurisma da Aorta Torácica/cirurgia , Ruptura Aórtica/cirurgia , Síndrome de Behçet/complicações , Implante de Prótese Vascular , Procedimentos Endovasculares , Adulto , Anestesia Local , Aneurisma da Aorta Torácica/diagnóstico por imagem , Aneurisma da Aorta Torácica/etiologia , Ruptura Aórtica/diagnóstico por imagem , Ruptura Aórtica/etiologia , Aortografia/métodos , Síndrome de Behçet/diagnóstico , Prótese Vascular , Implante de Prótese Vascular/instrumentação , Angiografia por Tomografia Computadorizada , Emergências , Procedimentos Endovasculares/instrumentação , Humanos , Masculino , Embolia Pulmonar/etiologia , Stents , Resultado do TratamentoRESUMO
Behçet's disease is an inflammatory systemic disorder, characterized by a relapsing and remitting course, it manifests with oral and genital ulcerations, skin lesions, uveitis, vasculitis, central nervous system and gastrointestinal involvement. The main histopathological finding is widespread vasculitis of the arteries and veins. Therapy is variable and depends largely on the severity of the disease and organ involvement. There is common practice to treat with anticoagulation in patients suffering from vessel thrombosis, but there are no control trials to support this tendency. Anticoagulation treatment can cause major bleeding events in patients suffering from aneurysms. In this case report we describe a treatment dilemma in a patient suffering from deep vein thrombosis and pulmonary aneurysms.
Assuntos
Síndrome de Behçet , Ciclofosfamida/administração & dosagem , Glucocorticoides/administração & dosagem , Hemorragia , Conduta do Tratamento Medicamentoso , Ativador de Plasminogênio Tipo Uroquinase/administração & dosagem , Trombose Venosa , Varfarina , Adolescente , Aneurisma/diagnóstico por imagem , Aneurisma/etiologia , Aneurisma/terapia , Anticoagulantes/administração & dosagem , Anticoagulantes/efeitos adversos , Síndrome de Behçet/complicações , Síndrome de Behçet/diagnóstico , Síndrome de Behçet/fisiopatologia , Síndrome de Behçet/terapia , Terapia Biológica/métodos , Fibrinolíticos/administração & dosagem , Hemorragia/induzido quimicamente , Hemorragia/prevenção & controle , Humanos , Imunossupressores/administração & dosagem , Masculino , Artéria Pulmonar/patologia , Radiografia , Prevenção Secundária/métodos , Índice de Gravidade de Doença , Resultado do Tratamento , Ultrassonografia , Trombose Venosa/diagnóstico por imagem , Trombose Venosa/tratamento farmacológico , Trombose Venosa/etiologia , Varfarina/administração & dosagem , Varfarina/efeitos adversosRESUMO
The arterial lesions affect about 10% of patients with Behçet's disease (BD). Aortic inflammatory involvement includes predominantly aortic aneurysmal lesions affecting most often the abdominal aorta. They account for the severity of the disease and are a leading cause of death when they hit the aorta or pulmonary arteries. Within the arterial lesions of BD, aortic involvement is, with femoral lesions, the most common site involved (18-28% of patients with vascular disease). Unlike other large vessels vasculitis (i.e. giant cell arteritis and Takayasu's arteritis) diffuse aortitis is observed in less than 5% of patients with BD. Aortic lesions of BD may be asymptomatic (systematic imaging or occasionally associated with other vascular event) or be revealed by the occurrence of abdominal, thoracic or lumbar pain, or an aortic valve insufficiency. Fever is frequently associated. Increase in acute phase reactants is common in these patients. Histological analysis may show infiltration by lymphocytes, neutrophils and plasma cells in the media and adventitia and a proliferation of the vasa vasorum in the media as well as a fibroblastic proliferation. In the later phase, a fibrous thickening of the media and adventitia is observed as well as a proliferation and thickening of the vasa vasorum. The therapeutic management should always include a medical treatment for the control of inflammation (corticosteroids, immunosuppressive drugs and/or biotherapy) and often an endovascular or surgical treatment if the aneurysm is threatening. The choice between endovascular or surgical treatment is considered case by case, depending on the experience of the team, anatomical conditions and of the clinical presentation. In this review, we provide a detailed and updated review of the literature to describe the aortic inflammatory damage associated with Behçet's disease.
Assuntos
Aortite/complicações , Síndrome de Behçet/complicações , Aneurisma Aórtico/diagnóstico por imagem , Aneurisma Aórtico/etiologia , Aneurisma Aórtico/terapia , Aortite/diagnóstico por imagem , Aortite/terapia , Síndrome de Behçet/diagnóstico por imagem , Síndrome de Behçet/terapia , Terapia Biológica/métodos , Arterite de Células Gigantes/diagnóstico por imagem , Arterite de Células Gigantes/etiologia , Arterite de Células Gigantes/terapia , Humanos , Imunossupressores/uso terapêutico , Arterite de Takayasu/diagnóstico por imagem , Arterite de Takayasu/etiologia , Arterite de Takayasu/terapiaRESUMO
BACKGROUND/AIMS: Anemia in patients with inflammatory bowel disease significantly affects the quality of life. The aim of this study was to investigate the frequency of and risk factors for anemia and to describe the management of anemia in patients with intestinal Behçet's disease (BD) in actual clinical practice. METHODS: We included 64 patients with intestinal BD who visited the outpatient clinic of a tertiary referral center in June 2011 and had available laboratory data for the subsequent 6 months. RESULTS: Anemia was detected in 26 patients (40.6%). After 6 months, anemia was still present in 14 of these patients (53.8%). The cause of anemia was investigated in eight patients (30.8%), and oral iron supplementation was prescribed to four patients (15.4%). Of these four patients, two (50%) recovered completely within 6 months. Anemia was associated with a high Disease Activity Index for Intestinal Behçet's Disease (DAIBD, p=0.024), erythrocyte sedimentation rate (p=0.003), and C-reactive protein (p=0.049) in univariate analysis. In multivariate analysis, the factor predictive for anemia in patients with intestinal BD was a higher DAIBD (≥40; odds ratio, 4.08; 95% confidence interval, 1.21 to 13.71; p=0.023). CONCLUSIONS: Although anemia is common in intestinal BD patients, its clinical importance is overlooked in daily practice. Moderate to severe disease activity is predictive of anemia.
Assuntos
Anemia/etiologia , Síndrome de Behçet/complicações , Enteropatias/complicações , Adulto , Anemia/tratamento farmacológico , Anemia/epidemiologia , Síndrome de Behçet/sangue , Síndrome de Behçet/patologia , Sedimentação Sanguínea , Proteína C-Reativa/análise , Suplementos Nutricionais , Gerenciamento Clínico , Feminino , Humanos , Enteropatias/sangue , Enteropatias/patologia , Ferro/uso terapêutico , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Razão de Chances , Valor Preditivo dos Testes , Fatores de Risco , Índice de Gravidade de Doença , Oligoelementos/uso terapêuticoRESUMO
Biotherapies used in clinical practice for the treatment of uveitis include monoclonal antibodies and fusion proteins (anti-TNFα, anakinra, tocilizumab and rituximab), interferons (IFN) and intravenous immunoglobulins (IVIg). IFN is capable of inducing prolonged remission and continued after his discontinuation, in 20-40% of patients. Side effects (flu-like, psychological effects) limit its use in practice. Anti-TNFα (infliximab and adalimumab) represents an attractive alternative therapeutic in severe uveitis refractory to immunosuppressants, especially in Behçet's disease. They are generally (>90% of cases) and rapidly effective but their action is often suspensive. Anti-TNFα requires an extended prescription or takes over from another immunosuppressant once ocular inflammation has been controlled. IVIg are used for the treatment of Birdshot's disease. Tolerance of IVIg is good but their efficacy is transient. Rituximab showed an efficacy in few observations of various inflammatory eye diseases (uveitis, scleritis and idiopathic inflammatory pseudo-tumors or associated with granulomatosis with polyangiitis) and cicatricial pemphigoid. The risk of infection limits its use in refractory diseases to conventional therapy. Anakinra (a soluble antagonist of IL-1r) showed interesting results in terms of efficiency in one small open study in Behçet's disease. Its safety profile is good and with a quick action that could be interesting for the treatment of severe uveitis.
Assuntos
Produtos Biológicos/uso terapêutico , Uveíte/tratamento farmacológico , Anticorpos Monoclonais Humanizados/uso terapêutico , Síndrome de Behçet/complicações , Síndrome de Behçet/tratamento farmacológico , Terapia Biológica/métodos , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Imunossupressores/uso terapêutico , Rituximab/uso terapêutico , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Fator de Necrose Tumoral alfa/imunologia , Uveíte/etiologiaRESUMO
AGEs are permanently modified macromolecule derivatives that form through nonenzymatic glycation of amino groups of proteins. Glycer-AGEs are highly toxic and play an important role in the pathogenesis of chronic inflammatory diseases. However, the contribution of glycer-AGEs to the pathogenesis of uveitis is unclear. In this study, we measured serum levels of glycer-AGEs in 100 patients with endogenous uveitis (22 with HLA-B27-associated uveitis, 20 with VKH disease, 14 with Behçet's disease, and 44 with sarcoidosis) and 33 healthy volunteers. We then examined the effect of the AGE inhibitor in a mouse model of human endogenous uveitis (EAU) by continuous oral administration of pyridoxamine at 200 or 400 mg/kg/day. Regardless of the etiology, serum glycer-AGE levels were significantly higher in patients with uveitis than in healthy subjects. Treatment with 400 mg/kg pyridoxamine significantly reduced the clinical and histological severity of EAU and was accompanied by a significant decrease in serum and retinal glycer-AGE levels and suppression of translocation of NF-κB p65 into the nucleus of retinal cells. Serum glycer-AGE levels may therefore serve as a biomarker of human uveitis, as well as systemic inflammation, and may contribute to the progression of uveitis, including diabetic iritis, via the activation of NF-κB.
Assuntos
Doenças Autoimunes/tratamento farmacológico , Produtos Finais de Glicação Avançada/antagonistas & inibidores , Piridoxamina/uso terapêutico , Retinite/tratamento farmacológico , Uveíte/tratamento farmacológico , Administração Oral , Adulto , Sequência de Aminoácidos , Animais , Doenças Autoimunes/sangue , Doenças Autoimunes/patologia , Síndrome de Behçet/sangue , Síndrome de Behçet/complicações , Modelos Animais de Doenças , Avaliação Pré-Clínica de Medicamentos , Proteínas do Olho/imunologia , Proteínas do Olho/metabolismo , Proteínas do Olho/toxicidade , Feminino , Antígeno HLA-B27/imunologia , Humanos , Masculino , Camundongos , Pessoa de Meia-Idade , Dados de Sequência Molecular , Fragmentos de Peptídeos/imunologia , Fragmentos de Peptídeos/toxicidade , Transporte Proteico/efeitos dos fármacos , Piridoxamina/administração & dosagem , Piridoxamina/farmacologia , Retina/metabolismo , Retinite/sangue , Retinite/etiologia , Retinite/patologia , Proteínas de Ligação ao Retinol/imunologia , Proteínas de Ligação ao Retinol/toxicidade , Sarcoidose/sangue , Sarcoidose/complicações , Uveíte/sangue , Uveíte/etiologia , Uveíte/patologia , Síndrome Uveomeningoencefálica/sangue , Síndrome Uveomeningoencefálica/complicaçõesRESUMO
Behçet's disease (BD) is a chronic systemic inflammatory disorder of unknown etiology with variable clinical manifestations. HLA-B51 allele is the most strongly associated known genetic factor. The mucocutaneous lesions (oral aphthae, genital aphthae, skin lesions such as pseudofolliculitis) constitute the hallmark of the disease, but also gastrointestinal, vascular, central nervous systems, and others may be involved. We report a case of a young man affected with Behçet's disease who presented facial telangiectasias and striae rubra in the inner region of his arms and at the level of his hips, as uncommon minor superficial vascular manifestations of BD. To manage them we have subjected the patient to a cycle of Intense Pulsed Light (IPL) therapy. Our findings showed that the use of IPL is a safe and effective treatment for telangiectasias and striae rubra, also in the complex clinical condition of Behçet's disease. In fact, the treatments were well tolerated, no sign of scarring or hyper/hypopigmentation was reported and we obtained a significant improvement of the lesions in terms of color and size of them.
Assuntos
Síndrome de Behçet/complicações , Técnicas Cosméticas/instrumentação , Terapia com Luz de Baixa Intensidade/instrumentação , Telangiectasia/etiologia , Telangiectasia/radioterapia , Adulto , Humanos , MasculinoAssuntos
Síndrome de Behçet/complicações , Síndrome de Behçet/terapia , Produtos Biológicos/efeitos adversos , Terapia Biológica/efeitos adversos , Infecções/complicações , Adalimumab , Adulto , Anticorpos Monoclonais/efeitos adversos , Anticorpos Monoclonais Humanizados/efeitos adversos , Feminino , Humanos , Infecções/etiologia , Inflamação , Infliximab , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Projetos de Pesquisa , Risco , Fatores de TempoRESUMO
La enfermedad de Behçet es una enfermedad inflamatoria multisistémica crónica que evoluciona por brotes. Es más común en Asia y en los países de la cuenca mediterránea oriental (Ruta de la Seda). En España la prevalencia es de 5 a 10 casos por 100.000 habitantes. Es una enfermedad de difícil diagnóstico por las numerosas y variadas manifestaciones clínicas y porque no se dispone de pruebas de laboratorio patognomónicas. El retraso en el diagnóstico, frecuente en países de baja prevalencia como España, aumenta la morbilidad y la mortalidad de los pacientes con enfermedad de Behçet (AU)
Behçet's disease is an inflammatory multisystemic chronic disease that progresses by outbreaks. It is more common in Asia and countries in the eastern Mediterranean basin (Silk Route). In Spain the prevalence is between 5 and 10 cases per 100,000 inhabitants. It is a difficult disease to diagnose because of the multiple and varied clinical manifestations, and because there are not pathognomonic laboratory tests available. The delay in the diagnosis, which is frequent in countries of low prevalence like Spain, increases the morbidity and the mortality of patients with Behçetìs disease (AU)
Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Síndrome de Behçet/complicações , Estomatite Aftosa/complicações , Eritema Nodoso/complicações , Indometacina/uso terapêutico , Omeprazol/uso terapêutico , Colchicina/uso terapêutico , Prednisona/uso terapêutico , Úlcera/complicações , Combinação Amoxicilina e Clavulanato de Potássio/uso terapêutico , Levamisol/uso terapêutico , Síndrome de Behçet/fisiopatologia , Síndrome de Behçet/terapia , Úlcera/diagnóstico , Estomatite Aftosa/diagnóstico , Síndrome de Behçet/diagnóstico , Eritema Nodoso/diagnóstico , Atenção Primária à Saúde , Indicadores de Morbimortalidade , Diagnóstico Diferencial , Radiografia TorácicaRESUMO
INTRODUCTION: Behçet's disease is a multi-system vascular-inflammatory disease with possible involvement of the central nervous system. Lesions of the corpus callosum on MRI have been rarely reported in this disease. CASE REPORT: A 47-year-old woman was admitted for a sudden right hemiplegia and confusion revealing a Behcet's disease. MRI showed a pedonculo-thalamic lesion and a white matter hypersignals, which was suggestive of the disease. Besides, involvement of the corpus callosum was observed. CONCLUSION: This case demonstrates that Behcet's disease should be considered among diseases with corpus callosum involvement.
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Síndrome de Behçet/patologia , Corpo Caloso/patologia , Corticosteroides/uso terapêutico , Azatioprina/uso terapêutico , Síndrome de Behçet/complicações , Colchicina/uso terapêutico , Confusão/etiologia , Feminino , Hemiplegia/etiologia , Humanos , Imunossupressores/uso terapêutico , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Tegmento Mesencefálico/patologia , Tálamo/patologia , Falha de TratamentoRESUMO
BACKGROUND: To describe the clinical characteristics and evolution of a series of adult patients hospitalized for neuro-Behçet disease (NBD). METHODS: Consecutive patients admitted for NBD in a teaching hospital were retrospectively selected. Disability at discharge and during follow-up was graded with the modified Rankin Scale, and outcome classified as good or poor (grades 3-6). RESULTS: Twenty patients were included (M/F, 13/7). Mean age at NBD diagnosis was 36.3 years. Nineteen patients had other manifestations of Behçet disease (BD) before NBD developed, but only 7 met the complete diagnostic criteria for BD. Fever, headache, motor weakness, and cranial nerve palsy were each present in approximately 60% of patients. There was a low prevalence of behavioral changes (5%), seizures (5%), and sphincter incontinence (0%), and a relatively high prevalence of meningism (25%). Non-neurologic manifestations of BD were concurrently detected in 15 patients (75%). 80% had parenchymal involvement. Brain biopsies during 5 attacks showed perivascular lymphocytic infiltration with reactive astrocytosis, but no frank vasculitis. During a mean follow-up of 6.3 years per patient, 12 had at least one relapse. In total, there were 22 relapses; all but two were in the same location and were symptomatically similar in each patient. At the end of follow-up, 7 patients (35%) had a poor outcome, including 4 who died. CONCLUSION: Recording of previous manifestations of BD and a physical examination to detect concomitant systemic manifestations of BD may help establish an early diagnosis of NBD. Relapses frequently occurred in the same location. No frank vasculitis was present in brain biopsies.