scholarly journals Immunopathogenesis of Ocular Behçet’s Disease

2014 ◽  
Vol 2014 ◽  
pp. 1-13 ◽  
Author(s):  
Un Chul Park ◽  
Tae Wan Kim ◽  
Hyeong Gon Yu
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Retinal Vasculitis ◽  
Skin Lesions ◽  
Unknown Etiology ◽  
Ocular Involvement ◽  
Inflammatory Disorder ◽  
General Hypothesis ◽  
Behcet's Disease

Behçet’s disease (BD) is a chronic recurrent systemic inflammatory disorder of unknown etiology characterized by oral and genital ulcerations, skin lesions, and uveitis. The ocular involvement of BD, or Behçet’s uveitis (BU), is characterized by panuveitis or posterior uveitis with occlusive retinal vasculitis and tends to be more recurrent and sight threatening than other endogenous autoimmune uveitides, despite aggressive immunosuppression. Although pathogenesis of BD is unclear, researches have revealed that immunological aberrations may be the cornerstone of BD development. General hypothesis of BD pathogenesis is that inflammatory response is initiated by infectious agents or autoantigens in patients with predisposing genetic factors and perpetuated by both innate and acquired immunity. In addition, a network of immune mediators plays a substantial role in the inflammatory cascade. Recently, we found that the immunopathogenesis of BU is distinct from other autoimmune uveitides regarding intraocular effector cell profiles, maturation markers of dendritic cells, and the cytokine/chemokine environment. In addition, accumulating evidence indicates the involvement of Th17 cells in BD and BU. Recent studies on genetics and biologics therapies in refractory BU also support the immunological association with the pathogenesis of BU. In this review, we provide an overview of novel findings regarding the immunopathogenesis of BU.

Clinical Findings, Diagnosis, and Classification of Behçet's Disease

2018 ◽  
pp. 329-333
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Retinal Vasculitis ◽  
Clinical Findings ◽  
Unknown Etiology ◽  
Ocular Involvement ◽  
Behcet's Disease ◽  
Diagnosis And Classification

Behçet’s Disease (BD) is a chronic multisystem vasculitis with unknown etiology, which is characterized by the triad of recurrent oral ulcers, genital ulcers, and iritis. The eye is one of the most commonly affected organs and typical ocular involvement is panuveitis with hypopyon and retinal vasculitis. The diagnosis is based on clinical evaluation including biomicroscopic and funduscopic examination. This article aims to review the clinical findings, diagnosis, and classification of BD.

Intraocular Surgery in Behcet's Disease

2018 ◽  
pp. 380-383
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Retinal Vasculitis ◽  
Skin Lesions ◽  
Biologic Agents ◽  
Posterior Segment ◽  
Ocular Involvement ◽  
Vitreoretinal Interface ◽  
Behcet's Disease

Behçet’s disease (BD) is a multisystemic vasculitis characterized by multiorgan involvement presenting with recurrent oral and/or genital ulcerations, skin lesions, and uveitis. Ocular involvement often occurs as remitting panuveitis and retinal vasculitis and the visual prognosis is poor if not treated properly. Relapsing uveitis attacks and chronic steroid administration during the treatment period may increase the risk of cataract and glaucoma formation. Also, recurrent uveitis may lead to severe visual compromise due to several posterior segment complications such as vitreous opacification, vitreoretinal interface changes, retinal neovascularization, cystoid macular edema, and optic atrophy. Systemic drugs including steroids, immunosuppressive, and biologic agents usually decrease the severity and frequency of the uveitis. Cataract, glaucoma, and vitreoretinal surgeries in eyes with uveitis may show some difference from the general practice. In this manuscript, approaches in anterior and posterior segment ocular surgeries in BD patients are reviewed.

scholarly journals Old and New Challenges in Uveitis Associated with Behçet’s Disease

2021 ◽  
Vol 10 (11) ◽  
pp. 2318
Author(s):  
Julie Gueudry ◽  
Mathilde Leclercq ◽  
David Saadoun ◽  
Bahram Bodaghi
Keyword(s):  
Behçet’S Disease ◽  
Structural Damage ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Systemic Vasculitis ◽  
Retinal Vasculitis ◽  
Unknown Origin ◽  
Current Data ◽  
Ocular Involvement ◽  
Behcet's Disease

Behçet’s disease (BD) is a systemic vasculitis disease of unknown origin occurring in young people, which can be venous, arterial or both, classically occlusive. Ocular involvement is particularly frequent and severe; vascular occlusion secondary to retinal vasculitis may lead to rapid and severe loss of vision. Biologics have transformed the management of intraocular inflammation. However, the diagnosis of BD is still a major challenge. In the absence of a reliable biological marker, diagnosis is based on clinical diagnostic criteria and may be delayed after the appearance of the onset sign. However, therapeutic management of BD needs to be introduced early in order to control inflammation, to preserve visual function and to limit irreversible structural damage. The aim of this review is to provide current data on how innovations in clinical evaluation, investigations and treatments were able to improve the prognosis of uveitis associated with BD.

scholarly journals A Darwinian view of Behçet's disease

2021 ◽  
Vol 2 (2) ◽  
pp. 91-99
Author(s):  
Rhodri Smith ◽  
Robert J. Moots ◽  
Mariam Murad ◽  
Graham R. Wallace
Keyword(s):  
Behçet’S Disease ◽  
Complex Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Clinical Findings ◽  
Unknown Etiology ◽  
Inflammatory Disorder ◽  
Protective Mechanisms ◽  
Behcet's Disease ◽  
Cns Lesions

Abstract Behçet’s disease (BD) is a multisystem inflammatory disorder of unknown etiology, characterized by oral and genital ulceration, with other complications including eye, skin, joint, and central nervous system (CNS) lesions. Diagnosis is based on clinical findings, which may differ between patients. There is a strong genetic basis for BD; however, only a few genes have been associated with the disease across the geographical spread of BD. In this article, we discuss the history and combination of genes involved in this complex disease in relation to the geographical range and present our view that the disease has developed from a Darwinian perspective, with different gene polymorphisms that affect the same biological pathway. Moreover, these mutations individually are protective mechanisms against the disease relevant to each region, which affected both archaic and modern humans.

THROMBOSES IN Behcet's DISEASE: FROM PATHOGENESIS TO TREATMENT

2018 ◽  
Author(s):  
З.C. Алекберова ◽  
Т.А. Лисицына ◽  
Р.Г. Голоева ◽  
А.В. Волков ◽  
Р.А. Осипянц ◽  
...  
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Erythema Nodosum ◽  
Behçet's Disease ◽  
Retinal Vasculitis ◽  
Vascular Pathology ◽  
Coagulation System ◽  
Unknown Etiology ◽  
Behcet's Disease ◽  
Pathergy Test

Введение. Болезнь Бехчета (ББ) – системный васкулит неизвестной этиологии, характеризующийся рецидивами язвенного процесса в ротовой полости и на гениталиях, частым поражением глаз и рядом проявлений, включающих патологию суставов, желудочно-кишечного тракта, нервной системы и сосудов. Цель исследования: определить частоту и локализацию сосудистых проявлений у больных с ББ, уточнить связь последних с полом, возрастом и другой клинической симптоматикой ББ. Материалы и методы. Обследовано 250 больных ББ, сосудистая патология выявлена у 63 (25,2%) пациентов. Возраст больных с сосудистыми проявлениями составил 34,8 ± 10,4 лет, соотношение мужчины:женщины – 3,5:1. Сосуды вовлекались в процесс чаще в течение первых трех лет болезни. Поражалось в основном венозное русло, доля больных с артериальной патологией составила 9,5%. Результаты. У пациентов с сосудистой патологией по сравнению с больными без тромбозов достоверно чаще выявлялись узловатая эритема (71% против 44%; р = 0,002) и эпидидимит (22,4 % против 10,9%; р = 0,04), а генерализованный увеит (17,4% против 32%; р = 0,002) и ангиит сетчатки (22% против 40,6%; р = 0,008) встречались достоверно реже. В системе коагуляции у больных ББ с и без сосудистой патологии достоверных различий не выявлено. Заключение. Сосудистые нарушения выявлены у ¼ больных (преимущественно мужчин молодого возраста), развивались чаще в первые три года болезни и ассоциировались с узловатой эритемой, эпидидимитом и положительным тестом патергии. Introduction. Behcet's disease (BB) is a systemic vasculitis of unknown etiology; it is characterized by recurrent aphthous stomatitis, genital ulcers, uveitis and other manifestations including arthritis, pathology of gastrointestinal tract, nervous system and blood vessels. The aim of study: to determine frequency and localization of vascular manifestations in patients with BB, to clarify the relationship of manifestations with sex, age and other clinical symptoms of BB. Materials and methods. We examined 250 patients with BB, vascular pathology was revealed in 63 (25,2%) patients. The age of patients with vascular manifestations was 34,8 ± 10,4 years, the ratio of men:women was 3,5:1. Vessels were involved in the process more often during the first three years of the disease. Mainly veins were affected, arterial pathology had 9,5% of patients. Results. In patients with vascular pathology erythema nodosum (71% vs. 44%, p = 0,002) and epididymitis (22,4% vs. 10.9%; p = 0,04) were significantly more frequent than in patients without thromboses, and panuveitis (17,4% vs. 32%; p = 0,002) and retinal vasculitis (22% vs. 40,6%; p = 0,008) were found significantly more rarely. There were no significant differences in coagulation system between patients with and without vascular pathology. Conclusion. Vascular disorders were detected in ¼ of patients (predominantly in men of young age), developed more often in the first three years of the disease and associated with erythema nodosum, epididymitis and a positive pathergy test.

Epidemiology, Prevalence, Incidence in Behçet's Disease

2018 ◽  
pp. 321-323
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Systemic Vasculitis ◽  
Skin Lesions ◽  
Medical Community ◽  
Aphthous Ulcer ◽  
Ocular Involvement ◽  
Behcet's Disease ◽  
Ancient Silk Road

Behçet’s disease (BD) was first brought to the attention of the modern medical community by Hulusi Behçet in 1937. The disease is a systemic vasculitis characterized by a recurrent aphthous ulcer, genital ulcer, uveitis, and skin lesions. It is observed more common and severe throughout the ancient Silk Road from East Asia to the Mediterranean. Ocular involvement is reported in 25-75% of patients with BD. In most cases, it may progress to blindness if left untreated. The aim of this review is to investigate the epidemiology, prevalence, and incidence of BD.

scholarly journals Histopathological Evaluation of Behçet's Disease and Identification of New Skin Lesions

2012 ◽  
Vol 2012 ◽  
pp. 1-7 ◽  
Author(s):  
Özgür Gündüz
Keyword(s):  
Differential Diagnosis ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Skin Lesions ◽  
Direct Immunofluorescence ◽  
Inflammatory Disorder ◽  
Behcet's Disease ◽  
Histopathological Evaluation ◽  
New Skin

Behçet's disease (BD) is a multisystemic, relapsing inflammatory disorder with an obscure etiology and pathogenesis. Diagnosis depends on the clinician's ability to identify a group of nonspecific mucocutaneous lesions, which also manifest in a number of other diseases. In recent years, there has been an increase in the studies focusing on the histopathological aspects of Behçet's disease diagnostic mucocutaneous lesions. Their results emphasize the value of histopathology and direct immunofluorescence (DIF) in the differential diagnosis of Behçet's disease.

A change in Ocular Involvement in a Patient Suffering from Ankylosing Spondylitis and Behçet's Disease

1995 ◽  
Vol 5 (3) ◽  
pp. 192-194 ◽  
Author(s):  
I. Beiran ◽  
J. Scharf ◽  
D. Dori ◽  
B. Miller
Keyword(s):  
Ankylosing Spondylitis ◽  
Behçet’S Disease ◽  
Anterior Uveitis ◽  
Clinical Picture ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
English Literature ◽  
Retinal Vasculitis ◽  
Ocular Involvement ◽  
Behcet's Disease

We report a case of a young man suffering from both ankylosing spondylitis and Behçet's disease in whom the character of the ocular involvement changed according to the predominant disease at a given time. When the clinical picture was one of ankylosing spondylitis, only anterior uveitis was observed, while the clinical picture of Behçet's disease occurred with panuveitis and retinal vasculitis. To the best of our knowledge, this is the first report in the English literature of two different patterns of ocular involvement in the same patient with two seronegative arthritides.

scholarly journals A Case of HLA-B51 Positive Mucocutaneous Variant of Behçet’s Disease in a Young Indian Male: A Case Report

2021 ◽  
Vol 2 (1) ◽  
pp. 37-43
Author(s):  
Ishan Sen ◽  
Debjani Majumder
Keyword(s):  
Case Report ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Indian Subcontinent ◽  
Unknown Etiology ◽  
Inflammatory Disorder ◽  
Pharyngeal Wall ◽  
Behcet's Disease ◽  
Indian Male

Background: Behçet’s disease is a rare, systemic inflammatory disorder of unknown etiology affecting the mucocutaneous, vascular, skeletal, ophthalmic, gastrointestinal, and neurological systems. While the exact etiopathogenesis of Behçet’s disease is yet to be established, numerous studies have supported a strong possibility of underlying genetic factors. It is comparatively more common in Turkey, the Middle East, and Mediterranean regions, and only a few cases have been reported from the Indian subcontinent so far. Although several immunological and genetic associations have been suggested, the diagnosis of Behçet’s disease remains primarily clinical and of exclusion. Case Report: In this report, we describe the case of a 22-year-old Indian male who developed multiple aphthous ulcers over his tonsillar pillars and pharyngeal wall following an episode of acute tonsillopharyngitis. Over the course of the next few days, he reported the presence of a genital ulcer and papulopustular lesions over his chest, back and face, eventually prompting a diagnosis of Behçet’s disease. He was treated with oral colchicine and was found to be in remission during a six-week follow-up. Conclusion: This case emphasizes the importance of awareness regarding Behçet’s disease among clinicians in India and warrants further studies on the epidemiology, immunopathogenesis, and management protocols of Behçet’s disease, especially in this country for a better understanding of its prevalence, manifestations, and disease course.

scholarly journals Behçet’s disease: Intracardiac thrombosis (a description of two cases and a review of literature)

2017 ◽  
Vol 89 (5) ◽  
pp. 79-82
Author(s):  
Z S Alekberova ◽  
P S Ovcharov ◽  
T A Lisitsyna ◽  
A V Volkov ◽  
T V Popkova
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Vena Cava ◽  
Skin Lesions ◽  
Left Ventricular ◽  
Aphthous Stomatitis ◽  
Unknown Etiology ◽  
Behcet's Disease ◽  
Intracardiac Thrombosis

Behçet’s disease (BD) is systemic vasculitis of unknown etiology, which is more common in the countries located along the Great Silk Road. The disease is diagnosed if a patient has 4 key diagnostic signs: aphthous stomatitis, genital sores, and eye and skin lesions. Vascular diseases referred to as minor criteria for BD are characterized by the formation of aneurysms and thrombosis, predominantly in the venous bed. In venous disorders, a blood clot can form in any vessel, including caval, cerebral, pulmonary, and other veins. The paper describes two clinical cases of BD with intracardiac thrombosis. In one case, a 24-year-old male patient with a documented diagnosis of BD, echocardiography revealed a left ventricular spontaneous echo contrast phenomenon that disappeared due to immunosuppressive therapy. The other case was a 34-year-old female patient, in whom the diagnosis was based on the international disease criteria: aphthous stomatitis, skin lesions (pseudopustulosis, erythema nodosum), and genital sores. Computed tomographic angiography showed a 3.7×2.2-cm mass (thrombus) in the right atrium. In addition, blood clots were present in the hepatic and inferior vena cava. No abnormalities in the coagulation system were found in both cases.

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