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.

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)

Behçet's disease: review with emphasis on dermatological aspects

Abstract: Behçet's disease is a systemic vasculitis characterized by attacks of acute inflammation, which can affect almost every vascularized area of the body. There is a close correlation between the geographical distribution of HLA-B51 and its prevalence. In the etiopathogenesis there are indications of genetic susceptibility associated with environmental influence. Among the involved genes are those that encompass innate and adaptive immunities. Polymorphisms and epistatic interactions in several genes are described, as well as the presence of imbalance lineage between HLA-B51 and A (MICA). Herpes simplex and Streptococcus sanguinis may be important extrinsic factors. An increase of Th1 response and of IL-21 is observed. The production of IL-21 is positively related to Th17 cells and negatively to T-regs. The mucocutaneous manifestations are Behcet´s disease markers, and their earlier onset indicates a worse prognosis. Recurrent oral ulcers have varied sizes and arrangements, genital ulcers are recurrent, leaving scars, skin lesions are multivaried, and pathergy, although not so frequent, is important for the diagnosis. There are numerous attempts to validate indexes that can evaluate the disease activity and among them the Mucocutaneous Activity Index. This is a specific score that can help with therapeutic decisions and to reduce morbidity, but still lacks validation. The clinical manifestations of other organs are described as well as treatment options.

Inner and outer segment junction (IS/OS line) integrity in ocular Behçet's disease / Integridade da junção do segmento ınterno e externo (linha IS/OS) na doença de Behçet ocular

Purpose: In this study, we examined the spectral domain optical coherence tomography (OCT) findings of ocular Behçet's disease (OB) in patients with inactive uveitis. Specifically, we analyzed the inner and outer segment junction (IS/OS line) integrity and the effect of disturbed IS/OS line integrity on visual acuity. Methods: Patient files and OCT images of OB patients who had been followed-up between January and June of the year 2013 at the Dicle University Eye Clinic were evaluated retrospectively. Sixty-six eyes of 39 patients were included the study. Results: OCT examination of the patients with inactive OB revealed that approximately 25% of the patients had disturbed IS/OS and external limiting membrane (EML) line integrity, lower visual acuity (VA), and lower macular thickness than others. Linear regression analysis revealed that macular thickness was not an independent variable for VA. In contrast, the IS/OS line integrity was an independent variable for VA in inactive OB patients. Conclusion: In this study, we showed that the IS/OS line integrity was an independent variable for VA in inactive OB patients. Further prospective studies are needed to evaluate the integrity of the IS/OS line in OB patients. .

Objetivo: Neste estudo, examinamos os achados da tomografia de coerência óptica (OCT) de domínio espectral na doença de Behçet ocular (OB) em pacientes com uveíte inativa. Especificamente, analisamos a integridade da junção dos segmentos interno e externo (linha IS/OS) e o efeito da alteração da integridade da linha IS/OS na acuidade visual. Métodos: Avaliamos retrospectivamente os prontuários e as imagens de OCT dos pacientes com OB examinados entre janeiro e junho de 2013 na Dicle University Eye Clinic. Sessenta e seis olhos de 39 pacientes foram incluídos no estudo. Resultados: Análise das imagens de OCT dos pacientes com OB inativa revelou que aproximadamente 25% dos pacientes tinham alterações da integridade da linha IS/OS e da membrana limitante externa (ELM), e apresentavam acuidade visual (VA) e espessura macular menor do que os outros. A análise de regressão linear revelou que a espessura macular não é uma variável independente para VA. Por outro lado, a integridade da linha IS/OS foi uma variável independente para VA em pacientes com OB inativa. Conclusão: Neste estudo, nós mostramos que a integridade da linha IS/OS foi uma variável independente para a VA em pacientes com OB inativa. Precisamos de mais estudos prospectivos para avaliar a integridade da linha IS/OS em pacientes com OB. .

Are Soluble ST2 Levels in the Picogram or Nanogram Range in Serum of Healthy Subjects/Disease Patients?

No abstract available.

Dermatoses neutrofílicas: parte I / Neutrophilic dermatoses: part I

Os autores apresentam uma revisão das dermatoses neutrofílicas que possuem grande repercussão à saúde dos pacientes: síndrome de Sweet, pioderma gangrenoso, doença de Behçet e urticária neutrofílica. São discutidos, baseados nos resultados e conclusões de estudos relevantes publicados recentemente e na experiência dos autores, os principais aspectos clínicos, as importantes alterações histopatológicas e as opções para o manejo.

The authors present a review of neutrophilic dermatoses that have great impact on the health of patients: Sweet syndrome, pyoderma gangrenosum, Behçet's disease and neutrophilic urticaria. Major clinical aspects, histopathological changes and management options are discussed based on the results and conclusions of relevant studies recently published and on the authors' experience.

Angio-Behçet: a propósito de un caso clínico / Angio-Behçet: a case report

Se ha denominado Angio-Behçet, al conglomerado de fenómenos tromboembólicos en contexto de una Enfermedad de Behçet, que correspondería a una presentación atípica de esta patología. A veces se presenta como accidente vascular isquémico y trombosis venosa asociada a signos clásicos de Enfermedad de Behçet: Úlceras orales y genitales y uveítis. La patogénesis de esta enfermedad ha sido ampliamente estudiada en relación a posible origen genético, autoinmune o infeccioso. El tratamiento es difícil y frecuentemente tardío debido a que el diagnóstico es muy complejo y de gran reto para el clínico.

Angio-Behçet is called to the group of thromboembolic phenomena, associated to Behçet Disease and corresponds to an atypical manifestation of this disease. Sometimes it as an ischemic stroke with venous thrombosis associated to the classical signs of Behçet Disease: oral and genital ulcers and uveitis. The pathogenesis of this disease has been widely studied, with reference to a possible genetic, autoimmune or infectious origin. The treatment is difficult and is frequently late, because the diagnosis is very complex, being a great challenge for any clinician.

[Venous involvement in behcet's disease: a series of 28 cases]

Venous involvement is a rare and severe clinical feature in Behcet disease [ED]. We reported our experience with a rare and interesting subset of ED patients with venous involvement. From 1994 to 2008, twenty eight eases of venous ED were found amongst BD patients in the department of internal medicine of Habib Thameur Hospital. 45 episodes of venous thrombosis occurred in 28 patients. There were 25 men and 3 women the mean age when venous involvement manifested was diagnosed was about 32.25. Venous thrombosis occur in the first five years following the diagnosis of BD. Pericarditis was more frequently noted in patients with venous ED than others without vasculo-Behcet disease. In contrast digestive and rheumatologic symptoms were more likely observed in patients without vasculo-l3ehcet disease. Thirteen patients underwent corticosteroids. Relapses occurred more frequently in patients without corticosteroid therapy. Venous involvement is more frequently noted in vasculo-Behcet than arterial involvement. Its treatment should include anticoagulation in association with corticosteroids

Two cases of neuro-Behcet's disease mimicking cerebral tumor.

Two cases of neuro-Behcet's disease with isolated, solitary fronto-temporal and mesencephalic lesions respectively are reported. The cases were misdiagnosed as cerebral tumor. The postoperative outcome of the first patient was not satisfactory as he developed hemispheric edema. The second patient, with mesencephalic lesion, was treated only with corticosteroids, and the patient improved significantly. The lesion in this case resolved completely at six-month follow-up. We conclude that distinguishing the isolated solitary cerebral lesion of the Behcet's disease from a tumor may prevent surgical intervention.

The Clinical Significance of a Pathergy Reaction in Patients with Behcet's Disease

This study was done to evaluate the frequency, intensity, and specificity of a positive pathergy reaction (PR) in Behcet's disease (BD) patients, to clarify an association between the PR and the clinical features or disease severity, and to assess whether patients with pustule formation at the venous puncture site (PFVPS) without positive PR could be regarded as a positive reaction. The PR was tested in 64 BD patients, 74 disease controls, and 20 healthy controls. Venous PR was performed in 8 BD patients with PFVPS. Follow-up PR was done in 14 patients with positive reaction during inactive phase. The PR was positive in 35.9% of BD patients, in 1 patient among disease controls, and in none of healthy controls. The pustule formation was observed in one BD patient. There was no statistical significance between positive PR and the clinical variables. The mean clinical activity score of BD patients with positive PR was similar to patients with negative reaction. Venous PR was positive in 7 patients. The follow-up PR was positive in 2 patients during inactive phase. Conclusively the positive PR appeared to be specific for BD, and was not associated with the clinical variables or disease severity, but was usually found during active phase in cases with positive reaction. The PFVPS in patients with negative PR might be considered to be positive.

Early detection of thoracic vessels involvement in patients with behcet's disease having one episode of lower extremity deep vein thrombosis

To determine the risk of thoracic vessels involvement in patients with Behcet's disease [BD] presenting with lower extremity deep vein thrombosis [DVT]. The study was performed from August 1997 to April 1998, on 10 patients with BD, who had at least one episode of DVT in the lower extremities in their past or present illness. The diagnosis of DVT was based on physical examination and sonography and/or Doppler sonography. Medical history taking regarding pulmonary symptoms such as: cough, with or without sputum production, dyspnea, chest pain, and hemoptysis as well as physical examination, including vascular examination, was performed in all the cases. A new chest x-ray was taken from all 10 cases and following an informed consent, 7 patients had a high resolution CT scan [HRCT] of the chest. All of the 10 studied cases underwent thoracic vessels angiography using the intra-venous digital subtraction angiography [IVDSA] method. The analysis of data was done using Fisher's exact test and student T-Test. Out of the 10 studied cases, seven were male and three were female. Three patients had only one episode, while the other 7 had more than one episode of DVT in the lower extremities. The time interval from diagnosis of BD to the emergence of vascular lesions was 7.8 years. Regarding respiratory manifestations, 5 out of 10 cases were symptomatic and the rest were asymptomatic. Chest x-rays of 9 out of 10 cases were normal, while in one case changes in favor of pulmonary artery aneurysm [PAA], were observed. HRCT scans from the thoracic cage were done in 7 out of 10 cases, with 3 positive findings, [2 with pulmonary artery thrombosis [PAT] and 1 with superior vena cava obstruction [SVCO]]. On thoracic vessel IVDSA 6 cases with positive findings were detected [3 with PAA, 3 with SVCO, and 2 with pulmonary artery thrombosis], in both symptomatic and asymptomatic cases. [Fisher exact =0.4850][p value=0.05%, t [9] = 1.97, risk = 30%]. Gender and pulmonary symptomatology had no significant effect with respect to the results. Patients with [BD] with at least one episode of DVT in the lower extremities have a 30% risk of thoracic vessels involvement by non-invasive angiography using the IVDSA method. Hemoptysis is a late heralding sign for thoracic vessels involvement in BD patients with thrombophelebitis of the lower extremities. Therefore, we recommend radiological screening of thoracic vessels by IVDSA method in this group of BD patients, especially in areas with an increased prevalence of BD. Chest x-ray is not a good screening method for detection of thoracic vessels involvement although HRCT scan can be a helpful procedure for detection of thrombosis in thoracic vessels

Dermatologic manifestation of Behcet's disease

Dermatologic lesions in Behcet's disease are regarded as important manifestation for diagnosis. Various kinds of cutaneous lesions appear in patients with Behcet's disease. They present as erythema nodosum-like lesion, papulopustular eruption, erythema multiforme-like lesion, thrombophlebitis, skin ulcer, Sweet's syndrome-like lesion, bullous necrotizing vasculitis, and pyoderma gangrenosum. The hyperreactivity of the skin to any intracutaneous injection or needle prick, which is known as pathergy, is one clinically-unique feature of the disease.

Ophthalmologic manifestation of Behcet's disease

Loss of vision in Behcet's disease is one of the most common, as well as one of the most serious, of its varied manifestations. Total blindness may often be the eventual outcome. The underlying problem in the eye, as well as in other organ systems, is an occlusive vasculitis. Although the most common ocular symptom is that of iridocyclitis, the presence of retinovascular lesions, especially necrotizing retinitis, is well known but often obscured by the severity of the anterior reaction, which precludes a good view of the retina. While Behcet's disease is characterized by spontaneous remissions and relapses, no external factors have been found to explain the length of remissions. Blindness, which occurs in up to 25% of patients, is one of the major causes of permanent disability.

Etiopathology of Behcet's disease: immunological aspects

Behcet's disease is recognized as a systemic inflammatory disease of unknown etiology. The disease has a chronic course with periodic exacerbations and progressive deterioration. Previous reports have shown at least three major pathophysiologic changes in Behcet's disease; excessive functions of neutrophils, vasculitis with endothelial injuries, and autoimmune responses. Many reports suggested that immunological abnormalities and neutrophil hyperfunction may be involved in the etiology and the pathophysiology of this disease. HLA-B51 molecules by themselves may be responsible, in part, for neutrophil hyperfunction in Behcet's disease. T cells in this disease proliferated vigorously in response to a specific peptide of human heat shock protein (hsp) 60 in an antigen-specific fashion. T cells reactive with self-peptides produced Th1-like proinflammatory and/or inflammatory cytokines. This leads to tissue injury, possibly via delayed-type hypersensitivity reaction, macrophage activation, and activation and/or recruitment of neutrophils. These data shed new light on the autoimmune nature of Behcet's disease; molecular mimicry mechanisms may induce and/or exacerbate Behcet's disease by bacterial antigens that have activated T cells which are reactive with self-peptide(s) of hsp. This would lead to positive selection of autoreactive T cells in this disease.

Estudo de parâmetros clínico laboratoriais em pacientes portadores da doença de Behçet / Study of clinical and laboratorial parameters in patients with Behçet's disease

Este trabalho foi realizado de forma prospectiva com 40 pacientes portadores da doença de Behçet do Serviço de Uveíte do Hospital Säo Geraldo, Universidade Federal de Minas Gerais. Foi observada uma proporçäo de 67 por cento de mulheres, sendo a razäo homem/mulher de 1:2. A distribuiçäo étnica foi a seguinte: 43 por cento faiodermas, 42 por cento leucodermas e 15 por cento melanodermas. O critério de diagnóstico foi estabelecido segundo a Comissäo Japonesa de Estudo da Doença de Behçet, sendo que a ocorrência dos sinais maiores foi de 97,5 por cento de úlcera oral, 87,5 por cento de lesäo dermatológica, 85 por cento de lesäo genital e 82,5 por cento de lesäo ocular. O teste da patergia (hiper-reatividade cutânea por picada de agulha) foi positivo somente em 10,3 por cento dos casos. Todos os pacientes do grupo controle apresentaram resultados negativos. Os achados histopatológicos do local do teste da patergia mostraram vasculite linfocítica em 62 por cento dos casos, vasculite leucocitoclástica em 21 por cento e resultado inespecífico em 17 por cento. O grupo controle mostrou resultado inespecífico ou normal em 80 por cento dos casos e vasculite linfocítica em 20 por cento. Biópsias realizadas após sete dias mostraram vasculite linfocítica ou leucocitoclástica somente nos pacientes portadores da doença de Behçet. O HLA-B51 positivo foi encontrado em 56,4 por cento dos pacientes e somente em 2,6 por cento no grupo controle. Näo foi observada nenhuma correlaçäo entre o HLA-B51 e as variáveis clínicas.