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1.
BMC Surg ; 21(1): 164, 2021 Mar 25.
Artigo em Inglês | MEDLINE | ID: mdl-33765960

RESUMO

BACKGROUND: Prosthetic valve detachment is not rare after aortic valve replacement in Behcet's disease. However, destruction of the intervalvular fibrous body (IFB) due to Behcet's disease was rarely reported. CASE PRESENTATION: We report a case of 30 year-old woman, with valve detachment and IFB separation. The patient received aortic valve replacement three months ago. Her medical history included recurrent oral ulcers and cutaneous lesions. Finally, reoperation was performed and peri-operative steroid therapy was carried out. CONCLUSIONS: The case presented a rare complication of valve detachment in Behcet's disease.


Assuntos
Valva Aórtica , Síndrome de Behçet , Próteses Valvulares Cardíacas , Falha de Prótese , Adulto , Valva Aórtica/cirurgia , Síndrome de Behçet/complicações , Síndrome de Behçet/diagnóstico , Feminino , Próteses Valvulares Cardíacas/efeitos adversos , Humanos
2.
Ann R Coll Surg Engl ; 103(3): e94-e97, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33645285

RESUMO

Behçet's disease is a rare disease characterised by recurrent oral ulcers, with systemic manifestations including genital ulcers, ocular disease, skin lesions, gastrointestinal disease, neurologic disease, vascular disease and arthritis. Most clinical manifestations of Behçet's disease are believed to be due to vasculitis. The heterogeneous clinical spectrum is influenced by sex, ethnicity and country of residence. Vascular manifestation in the form of isolated large brachial artery aneurysm is rare in children. Treatment involves aneurysmorrhaphy to avoid rupture or ischaemic sequelae in addition to lifelong medical management to control vasculitis.


Assuntos
Aneurisma/diagnóstico por imagem , Síndrome de Behçet/diagnóstico , Artéria Braquial/diagnóstico por imagem , Trombose/diagnóstico por imagem , Aneurisma/etiologia , Aneurisma/patologia , Aneurisma/cirurgia , Anticorpos Antinucleares/imunologia , Síndrome de Behçet/complicações , Síndrome de Behçet/imunologia , Síndrome de Behçet/patologia , Sedimentação Sanguínea , Artéria Braquial/patologia , Artéria Braquial/cirurgia , Proteína C-Reativa/imunologia , Pré-Escolar , Angiografia por Tomografia Computadorizada , Antígeno HLA-B51/imunologia , Humanos , Masculino , Veia Safena/transplante , Trombose/etiologia , Trombose/patologia , Trombose/cirurgia , Enxerto Vascular/métodos
3.
Yonsei Med J ; 62(2): 149-158, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-33527794

RESUMO

PURPOSE: We investigated whether antineutrophil cytoplasmic antibody (ANCA) positivity is associated with vascular manifestations at diagnosis of Behçet's disease (BD) and poor outcomes during follow-up. MATERIALS AND METHODS: We retrospectively reviewed the medical records of 1060 patients with BD. Among them, 808 patients could be diagnosed with BD based on the revised version of the International Criteria for Behçet's Disease (ICBD) in 2014 (2014 ICBD criteria) and 588 patients could be diagnosed with BD based on the International Study Group (ISG) criteria proposed in 1990 (1990 ISG criteria). We examined the sites and patterns of vascular involvement in the BD patients at diagnosis and evaluated adverse outcomes during follow up, such as all-cause mortality, acute coronary syndrome, and deep vein thrombosis. RESULTS: Among the 808 patients with BD based on the 2014 ICBD criteria, the rate of ANCA positivity at diagnosis was 2.2%. ANCA-positive BD patients exhibited a higher frequency of overall vascular manifestations (22.2% vs. 6.1%) and higher frequencies of vascular involvement in the upper extremities and visceral arteries than ANCA-negative BD patients (5.6% vs. 0.1% and 5.6% vs. 0.1%). Among the 588 BD patients based on the 1990 ISG criteria, similarly, ANCA-positive BD patients exhibited a higher frequency of vascular manifestations than ANCA-negative BD patients. ANCA positivity, however, did not seem to be associated with poor outcomes in BD patients during follow up. CONCLUSION: ANCA positivity in BD patients was found to be associated with cross-sectional vascular involvement in the upper extremities and visceral arteries at diagnosis but was not predictive of poor outcomes during follow-up.


Assuntos
Anticorpos Anticitoplasma de Neutrófilos/imunologia , Síndrome de Behçet/imunologia , Vasos Sanguíneos/patologia , Adulto , Síndrome de Behçet/diagnóstico , Estudos Transversais , Feminino , Seguimentos , Humanos , Masculino , Avaliação de Resultados em Cuidados de Saúde , Estudos Retrospectivos
5.
Clin Exp Rheumatol ; 38 Suppl 127(5): 69-75, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33331268

RESUMO

OBJECTIVES: The objective of the present study was to assess the efficacy of apremilast (APR) in the management of refractory oral and/or genital ulcers in patients with Behçet's disease (BD). METHODS: National multicentre open-label observational study on BD patients with recurrent oral and/or genital ulcers. In all cases orogenital ulcers were refractory to conventional therapy. APR was given and maintained at standard dose of 30 mg twice daily. The main outcome was the achievement of oral and/or genital ulcers remission. Efficacy of APR for other clinical manifestations was also evaluated. RESULTS: We included 51 patients (35 women/16 men; mean age 44.7±13.2 years). Before APR, all patients had received several systemic conventional and/or biologic drugs. APR was initiated because of refractory oral (n=19) or genital (n=2) aphthous ulcers or both (n=30). Other manifestations found at APR onset were arthralgia/arthritis (n=16), folliculitis/pseudofolliculitis (n=14), erythema nodosum (n=3), furunculosis (n=2), paradoxical psoriasis induced by TNF-α-inhibitors (n=2), ileitis (n=2), deep venous thrombosis (n=2), leg ulcers (n=1), erythematosus and scaly skin lesions (n=1), fever (n=1), unilateral anterior uveitis (n=1) and neuro Behçet (n=1). After a mean follow-up of 8.5±6.9 months, most patients had experienced improvement of orogenital ulcers and prednisone dose had been successfully reduced or discontinued. APR also yielded improvement of some non-aphthous manifestations such as the cutaneous follicular and intestinal manifestations. However, the effect on musculoskeletal manifestations was variable. CONCLUSIONS: APR yielded a rapid and maintained improvement of refractory mucocutaneous ulcers of BD, even in patients refractory to several systemic drugs including biologic therapy.


Assuntos
Síndrome de Behçet , Estomatite Aftosa , Adulto , Síndrome de Behçet/complicações , Síndrome de Behçet/diagnóstico , Síndrome de Behçet/tratamento farmacológico , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Talidomida/efeitos adversos , Talidomida/análogos & derivados , Úlcera
6.
Clin Exp Rheumatol ; 38 Suppl 127(5): 80-85, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33331270

RESUMO

OBJECTIVES: Behçet's disease (BD) is characterised by repeated acute inflammatory attacks with aphthous ulcers of the oral mucosa, uveitis of the eyes, skin symptoms, and genital ulcers. Although its aetiology is still unknown, there is evidence of the involvement of oral bacteria in systemic diseases. Various types of oral bacteria may be involved in the development and progression of BD. The present study investigated alterations in the oral flora of patients with BD in Mongolia. We collected saliva samples from the Mongolian BD group and healthy control (HC) group, and the oral flora were analysed using next-generation sequencer (NGS). METHODS: DNA was extracted from the unstimulated saliva samples from the 47 BD and 48 HC subjects. The DNA was amplified from the V3-V4 region of 16S rRNA using PCR, and the data were acquired using NGS. Based on the obtained data, we analysed the alpha diversity, beta diversity, and bacterial taxonomy of the salivary flora. RESULTS: Beta diversity differed significantly between the BD and HC flora, but no significant differences were observed in alpha diversity. We found that the proportions of three genera - an S24-7 family unknown species, a mitochondria family unknown species, and Akkermansia species associated with IL-10 production - were significantly lower in the BD than in the HC group. CONCLUSIONS: The reduced proportions of the S24-7 family and symbiotic Akkermansia species may be key phenomena in the oral flora of patients with BD.


Assuntos
Síndrome de Behçet , Estomatite Aftosa , Bactérias/genética , Síndrome de Behçet/diagnóstico , Humanos , RNA Ribossômico 16S/genética , Saliva
7.
Clin Exp Rheumatol ; 38 Suppl 127(5): 3-10, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33331271

RESUMO

Behçet's syndrome is a variable vessel vasculitis with multi-system involvement that shows important heterogeneity among patients regarding demographic features, organ manifestations, frequency and severity of relapses, disease course, response to treatment and prognosis. This heterogeneity has made it difficult to interpret and compare the results of studies, to standardise disease assessment and to develop management strategies. Several new studies have been published during the previous year exploring the epidemiology, pathogenesis, clinical manifestations, diagnosis, and management of Behçet's syndrome. The aim of this review is to provide an overview of the most relevant recent research in Behçet's syndrome from a critical perspective.


Assuntos
Síndrome de Behçet , Síndrome de Behçet/diagnóstico , Síndrome de Behçet/tratamento farmacológico , Síndrome de Behçet/epidemiologia , Progressão da Doença , Humanos , Prognóstico , Recidiva
9.
BMJ Case Rep ; 13(12)2020 Dec 17.
Artigo em Inglês | MEDLINE | ID: mdl-33334747

RESUMO

A 25-year-old Indian man presented with low-grade fever followed by gradually increasing swelling of neck and face. Physical examination showed bilateral neck swelling, facial swelling and dilated veins in the upper chest. Superior vena cava (SVC) obstruction due to an underlying malignancy was suspected. CT thorax showed large saccular aneurysm with thrombosis of bilateral subclavian arteries of which the right one caused external compression of right innominate vein draining into the SVC. A history of recurrent oral and scrotal ulcers was obtained following which skin pathergy test was done, which was suggestive of a diagnosis of Behcet's disease (BD). He responded to treatment with steroids and azathioprine. This report illustrates that rare nonmalignant cause such as BD could also present with SVC obstruction.


Assuntos
Aneurisma/diagnóstico , Síndrome de Behçet/diagnóstico , Artéria Subclávia/imunologia , Síndrome da Veia Cava Superior/diagnóstico , Adulto , Aneurisma/tratamento farmacológico , Aneurisma/imunologia , Anticoagulantes/administração & dosagem , Azatioprina/administração & dosagem , Síndrome de Behçet/sangue , Síndrome de Behçet/complicações , Síndrome de Behçet/imunologia , Proteína C-Reativa/análise , Glucocorticoides/administração & dosagem , Humanos , Imageamento Tridimensional , Masculino , Testes Cutâneos , Artéria Subclávia/diagnóstico por imagem , Síndrome da Veia Cava Superior/sangue , Síndrome da Veia Cava Superior/tratamento farmacológico , Síndrome da Veia Cava Superior/etiologia , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Veia Cava Superior/diagnóstico por imagem
12.
J Med Life ; 13(2): 164-169, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32742508

RESUMO

Behçet's disease is a systemic vasculitis. Mucocutaneous involvement is the most prominent finding, but triggering factors are not well-known. We decided to assess the beliefs of patients with Behçet's disease regarding the potential role of food, mucosal injury, menstruation, and stress in the appearance of symptoms. In this cross-sectional study, 60 patients with Behçet's disease who fulfilled the International Study Group criteria for Behçet's disease and referred to the outpatient Behçet's clinic of Motahari, affiliated to Shiraz University of Medical Sciences, were included. A questionnaire was designed by the research team consisting of the rheumatologist involved in the study, two dietitians, and a psychiatrist. The patients were interviewed face-to-face to fill in the questionnaire. The assessed variables were all food categories, menstruation, psychological stress, and oral mucosal injury as the potential triggers of symptoms onset. The most common foods reported as triggers for oral ulcers were eggplant (78.3%), melon (68.3%), walnut (68.2%), and cantaloupe (66.7%). Walnut was reported by three patients (5%) as the most common trigger for genital ulcers. Nervous tension (83%) and annoying arguments (45%) were the two most common psychological stress triggers for oral ulcers. Seven patients (11.7%) reported tooth brushing, as the trigger for oral ulcers. The irregular menstrual cycle was a trigger for oral ulcers in only two patients. Food items such as eggplant, walnut and melon were common self-reported triggers for mucocutaneous lesions in patients with Behçet's disease. Nervous tension and annoying arguments were also common psychological triggers for oral aphthous ulcers.


Assuntos
Síndrome de Behçet/psicologia , Cultura , Alimentos , Menstruação/psicologia , Mucosa Bucal/patologia , Estresse Psicológico/complicações , Adulto , Síndrome de Behçet/diagnóstico , Estudos Transversais , Feminino , Humanos , Masculino , Úlceras Orais/complicações , Úlceras Orais/psicologia , Dor/etiologia , Recidiva
15.
Laryngorhinootologie ; 99(6): 377-390, 2020 06.
Artigo em Alemão | MEDLINE | ID: mdl-32557459

RESUMO

Behçet's syndrome (BS) is classified as a variable vessel vasculitis. The clinical picture is very diverse and usually requires interdisciplinary collaboration. Pathogenetically, BS seems to take a middle position between a polygenic autoinflammatory disease and an autoimmune disease. New EULAR recommendations were issued in 2018. The therapy depends on which organs are most affected. Since 2016, adalimumab has been approved for the treatment of posterior ocular involvement. Infliximab, interferon a2a, interleukin-1 antagonists and apremilast may be alternative therapies.


Assuntos
Síndrome de Behçet , Síndrome de Behçet/diagnóstico , Síndrome de Behçet/tratamento farmacológico , Humanos
16.
Med. clín (Ed. impr.) ; 154(12): 488-492, jun. 2020. tab
Artigo em Espanhol | IBECS | ID: ibc-195675

RESUMO

INTRODUCCIÓN: Las manifestaciones neurológicas en la enfermedad de Behçet (EB) presentan una prevalencia variable en la literatura y asocian una mayor morbimortalidad. Se distingue la afectación parenquimatosa y la no parenquimatosa (vascular). El tratamiento de estas manifestaciones se basa en la inmunosupresión. La evidencia sobre la presentación y evolución del neurobehçet (NB) es escasa. MÉTODOS: Estudio observacional retrospectivo que incluyó a pacientes consecutivos con diagnóstico de EB entre 2006 y 2019 en un hospital terciario de Madrid. RESULTADOS: Se incluyó a 57 pacientes con EB, con un seguimiento medio de 7,83 (±6,98) años. La edad media al diagnóstico de EB y del primer episodio de NB fue 34,7 (±12,1) años y 31 (±9,9) años, respectivamente. Se registraron 56 episodios de NB en 25 pacientes. La fiebre (OR 3,39; IC 95%: 1,14-10,15) y la pseudofoliculitis (OR 4,06; IC 95%: 1,11-14,80) se asociaron a NB. La neuralgia del trigémino se encontró en el 16% (4/25) de los pacientes. CONCLUSIÓN: La prevalencia de NB fue mayor que la descrita previamente. La fiebre y la pseudofoliculitis se asociaron a NB y la mortalidad fue baja durante el seguimiento


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


Assuntos
Humanos , Masculino , Feminino , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Síndrome de Behçet/diagnóstico , Imunossupressão , Síndrome de Behçet/epidemiologia , Síndrome de Behçet/terapia , Estudos Retrospectivos , Foliculite/complicações , Neuralgia do Trigêmeo/complicações , Imunossupressores/uso terapêutico , Neuroimagem/métodos
19.
Isr Med Assoc J ; 22(5): 289-293, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32378820

RESUMO

BACKGROUND: Behçet's disease is a multi-systemic chronic relapsing inflammatory disease, classified among the vasculitides. The heterogeneity of clinical manifestations challenges the disease management. OBJECTIVES: To assess efficacy and safety of adalimumab in patients with active persistent Behçet's arthritis who did not respond to disease-modifying anti-rheumatic drugs and to assess the impact of treatment on the cytokine milieu. METHODS: Our cohort comprised 10 patients with active arthritis who received adalimumab in a 24-week investigator-initiated prospective open-label study. Patients who relapsed within 12 weeks following adalimumab discontinuation could enter a 3-year extension study. The patients underwent a comprehensive assessment including questionnaires and measurement of inflammatory cytokines, adalimumab serum levels, and anti-drug antibodies. RESULTS: A significant improvement was observed in arthritis, disease activity visual analogue scales, Behçet's disease current activity form, and interleukin-6 (IL-6) levels, but not in health assessment questionnaire and functional assessment of chronic illness therapy fatigue scale questionnaire. Resolution of oral and urogenital ulcers was achieved in all patients. Significant reduction of pain was reported by 40% of patients. The disease relapsed in 9 of 10 patients, within 2-6 weeks following adalimumab discontinuation. Of the 7 patients who continued the study, arthritis was resolved in 5. Two patients with high neutralizing antidrug antibodies titer relapsed. CONCLUSIONS: Adalimumab treatment achieved a significant improvement in arthritis, mucocutaneous manifestations, and IL-6 levels in all study patients but only 40% reported significant pain reduction. The arthritis relapsed in 90% of patients following adalimumab discontinuation and long-term treatment was required.


Assuntos
Adalimumab/uso terapêutico , Anti-Inflamatórios/uso terapêutico , Artrite/tratamento farmacológico , Síndrome de Behçet/tratamento farmacológico , Adalimumab/efeitos adversos , Adulto , Anti-Inflamatórios/efeitos adversos , Artrite/sangue , Artrite/etiologia , Síndrome de Behçet/sangue , Síndrome de Behçet/complicações , Síndrome de Behçet/diagnóstico , Citocinas/sangue , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Fatores de Tempo , Resultado do Tratamento
20.
Vascular ; 28(6): 829-833, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-32448079

RESUMO

OBJECTIVE: This study aims to investigate the efficacy and reliability of infliximab treatment in Behcet's disease with vascular involvement. METHODS: This single-center retrospective study included a total of 18 patients diagnosed with Behcet's disease with vascular involvement who were initiated infliximab treatment after exhibiting resistance to conventional immunosuppressive treatments. RESULTS: Seventeen patients achieved remission with infliximab treatment. While 18 patients were receiving a median of 50 (IQR: 20-61) mg/day equivalent of methylprednisolone before infliximab treatment, after infliximab treatment, only four patients were receiving 4 mg/day equivalent of methylprednisolone (p < 0.001). Only 4 patients were receiving oral anticoagulant treatment during infliximab treatment, and compared to the patients who were not receiving oral anticoagulants, there was no significant difference between the two groups according to occurrence of new vascular events. CONCLUSION: Infliximab seems to be an effective and reliable treatment in Behcet's disease with vascular involvement and may also allow reduced dosage or even the discontinuation of corticosteroids. The results of our study suggest that oral anticoagulant use is unnecessary in Behcet's disease with vascular involvement. However, further long-term randomized controlled studies are needed to investigate the length of infliximab regimen, whether or not it should be discontinued, and if so, whether or not immunosuppressants should be given as maintenance after discontinuation.


Assuntos
Síndrome de Behçet/tratamento farmacológico , Imunossupressores/uso terapêutico , Infliximab/uso terapêutico , Adulto , Idoso , Síndrome de Behçet/diagnóstico , Síndrome de Behçet/imunologia , Feminino , Humanos , Imunossupressores/efeitos adversos , Infliximab/efeitos adversos , Masculino , Pessoa de Meia-Idade , Indução de Remissão , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento , Turquia , Adulto Jovem
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