scholarly journals Behçet’s disease in clinical practice

2013 ◽  
Vol 94 (4) ◽  
pp. 545-548
Author(s):  
R F Khamitov ◽  
L Y Palmova ◽  
Z N Yakupova ◽  
E R Khasanova
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Retinal Vasculitis ◽  
Clinical Manifestations ◽  
Specific Treatment ◽  
Skin Lesions ◽  
Female Ratio ◽  
Behcet's Disease ◽  
Pathergy Test

Behçet’s disease is a chronic relapsing multisystemic vasculitis involving vessels of different diameter. The disease is characterized by involvement of skin and mucosa, eyes, gastrointestinal tract, joints, vessels, genitourinary system and neurological symptoms. According to data, male/female ratio is 2-10:1 The etiology of the disease is still unclear. Diagnosis is mainly based on thorough analysis of clinical manifestations. According to the International Study Group for Behçet’s Disease Diagnostic Criteria, combination of recurrent oral aphthosis and at least any two of the following: severe or scarring genital aphthosis, eye lesions (posterior uveitis, retinal vasculitis), skin lesions (nodular erythema, pseudofolliculitis, acne-like lesions) and positive pathergy test is needed to classify the patient as a patient with Behçet’s disease. Several clinical subtypes of Behçet’s disease are distinguished depending on predominant involvement of different organs and systems, including skin and mucosa, joints, eyes, or neurological manifestations. There are no specific laboratory tests for Behçet’s disease. The treatment approaches are variable depending on clinical manifestations. The effect of systemic corticosteroids, colchicine, azathioprine, chlorambucil, cyclophosphamide, sulfasalazine, methotrexate, cyclosporine, infliximab was proven. All the abovementioned requires the good clinical knowledge on Behçet’s disease from practicing doctors of different specialties, particularly - physicians. A case of highly active acute Behçet’s disease with nose, pharynx, eye and oral cavity mucosa, scrotum skin involvement is presented in the article. Questions of differential diagnosis are reviewed, specific treatment program with efficacy analysis is presented.

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.

Behçet's Disease from Rheumatology and Neurology Perspectives with Special Reference to Children

2019 ◽  
Vol 18 (04) ◽  
pp. 175-181
Author(s):  
Yaşar B. Turgut ◽  
Ayşe F. Tosun ◽  
Beste K. Yüzbaşı ◽  
Hasan Tunca
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
International Study ◽  
Skin Lesions ◽  
Disease Classification ◽  
Behcet's Disease ◽  
Male Predominance ◽  
Pediatric Age Group ◽  
Pathergy Test

AbstractBehçet's disease (BD) is an idiopathic chronic relapsing multisystemic vascular inflammatory disease. Although it is generally seen in young adults with male predominance, the onset age of the disease is the pediatric age group in only 4 to 26% of all cases of BD. According to the International Study Group for Behçet's Disease Classification Criteria, a diagnosis of BD requires recurrent oral aphthous ulcerations with two of the following: genital ulcerations, skin lesions, eye lesions, or a positive pathergy test. In this article, we extensively reviewed the current literature with respect to the rheumatological and neurological findings of BD in childhood.

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.

scholarly journals Diagnosis/Classification Criteria for Behcet's Disease

2012 ◽  
Vol 2012 ◽  
pp. 1-5 ◽  
Author(s):  
Fereydoun Davatchi
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Far East ◽  
Skin Lesions ◽  
Historical Background ◽  
Vascular Lesions ◽  
Behcet's Disease ◽  
Pathergy Test ◽  
Eye Lesions

Historical Background. The ISG criteria for Behcet's, created in 1990, have excellent specificity, but lack sensitivity. The International Criteria for Behcet's Disease (ICBD) was created in 2006, as replacement to ISG. The aim of this study was to compare their performance. ISG and ICBD Criteria. For ISG oral aphthosis is mandatory. The presence of any two of the following (genital aphthosis, skin lesions, eye lesions, and positive pathergy test) will diagnose/classify the patient as BD. For ICBD, vascular lesions were added, while oral aphthosis is no more mandatory. Getting 3 or more points diagnose/classify the patient as BD (genital aphthosis 2 points, eye lesions 2 points, and the remaining each one point). Performance and Comparison of ISG and ICBD. Their sensitivity, specificity, and accuracy (percent agreement), were tested in three independent cohort of patients from Far-East (China), Middle-East (Iran), and Europe (Germany). The sensitivity for ISG was respectively 65.4%, 78.1%, 83.7% and for ICBD 87%, 98.2%, and 96.5%. The specificity for ISG was 99.2%, 98.8%, 89.5% and for ICBD 94.1%, 95.6%, and 73.7%. The accuracy for ISG was 74.2%, 85.5%, 85.5% and for ICBD 88.9%, 97.3%, and 89.5%. Conclusion. ICBD has better sensitivity, and accuracy than ISG.

scholarly journals A rare coexistence of Behcet’s disease and Graves’ thyrotoxicosis in a young man: a case report

2020 ◽  
Vol 2020 (1) ◽  
Author(s):  
Hareesh Joshi ◽  
Imran Shahriar ◽  
Poonam Sharma ◽  
Satyanarayana V Sagi ◽  
Samson O Oyibo
Keyword(s):  
Behçet’S Disease ◽  
Inflammatory Markers ◽  
Behcet’S Disease ◽  
Clinical Symptoms ◽  
Behçet's Disease ◽  
Autoimmune Disorder ◽  
Skin Lesions ◽  
Behcet's Disease ◽  
Pathergy Test ◽  
Vasculitic Disorder

Abstract Behcet’s disease is a recurrent systemic vasculitic disorder. It manifests most commonly in the form of skin lesions, oral and genital ulcers and uveitis. Graves’ thyrotoxicosis is an autoimmune disorder characterized by excessive production of thyroid hormones. We present a case of a 41-year-old male of Turkish descent who had symptoms of arthralgia, rash, palpitations and weight loss. Bloods tests showed raised inflammatory markers and biochemical evidence of severe autoimmune thyrotoxicosis. The patient was HLA-B51-negative, and pathergy test was inconclusive. A diagnosis of Behcet’s disease was made on constellation of clinical symptoms. The patient was treated with carbimazole and prednisolone followed by azathioprine. The coexistence of Behcet’s disease and Graves’ disease in the same patient is very rare. Further studies are required to determine if there is a pathological association between these two conditions.

scholarly journals Isolated Aortitis with multiple Aortic aneurysms and widespread venous, arterial and intracardiac thrombosis: A challenging Vascular Behcet’s Disease case management

2021 ◽  
pp. 195
Author(s):  
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Clinical Manifestations ◽  
Skin Lesions ◽  
Sudden Onset ◽  
Aortic Aneurysms ◽  
Behcet's Disease ◽  
Disease Case ◽  
Intracardiac Thrombosis

In 1937, Hulusi Behcet’s brought to our attention the existence of a disease characterized by recurrent oral aphthae and any of several systemic manifestations including genital aphthae, ocular disease, skin lesions, gastrointestinal involvement, neurologic disease, vascular disease, or arthritis [1-3]. He became the first physician to describe this and the disease was henceforth named after his name. The aetiology of this disease remains unclear however it has been noticed that Behcet’s syndrome is remarkable for its systemic vasculitides which include its ability to involve blood vessels of all sizes (small, medium, and large) on both the arterial and venous sides of the circulation with occlusive nature [4]. This makes Vasculitis the major cause of the clinical manifestations of the disease with the involvement of almost any organ. We herein report an unusual case of a thirty-year-old Syrian man with no past medical illnesses. On presentation, he suffered from a sudden onset of shortness of breath and a history of hoarseness of voice for less than a week, and drastic weight loss of about thirty kgs within a month. Imaging confirmed the presence of pulmonary embolism, bilateral deep venous thrombosis, intracardiac thrombosis, popliteal artery thrombosis with thoracic, and abdominal aortic aneurysms. This case highlights the importance of having a high suspicion of the possibility of Behcet’s disease when multiple thrombi and aneurysms manifest in a patient on the silk root country. Thereby, stressing the importance of familiarizing ourselves with such unusual presentations of Behcet’s disease and the course of this unusual presentation with some learning points with a good literature review included below.

Plasma Homocysteine in Behcet's Disease: A Systematic Review and Meta-Analysis

2022 ◽  
Author(s):  
Mira Merashli ◽  
Tommaso Bucci ◽  
Daniele Pastori ◽  
Pasquale Pignatelli ◽  
Alessia Arcaro ◽  
...  
Keyword(s):  
Systematic Review ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Clinical Manifestations ◽  
Plasma Homocysteine ◽  
Vascular Involvement ◽  
Ocular Involvement ◽  
Female Ratio ◽  
Behcet's Disease

Abstract Aim To evaluate the relevance of plasma homocysteine (HC) in Behcet's disease (BD) and its clinical manifestations. Methods Systematic review of EMBASE and PubMed databases according to PRISMA guidelines from inception to July 2021; random-effects meta-analyses for continuous outcomes. Results The search strategy retrieved 48 case–control (2,669 BD and 2,245 control participants) and 5 cohort studies (708 BD participants). Plasma HC was higher in BD than in controls (p < 0.0001) with wide heterogeneity (I2  = 89.7%) that remained unchanged after sensitivity analysis according to year of article publication, age of BD participants, study size, study quality, method of HC determination, and male/female ratio >1.5; some pooled ethnicities explained a small part of the heterogeneity (I2  = 16.3%). Active BD participants had higher HC than inactive ones (p < 0.0001), with moderate heterogeneity (I2  = 49.2%) that disappeared after removal of an outlier study with very high disease activity. BD participants with any vascular involvement had higher HC than those without (p < 0.0001) with wide heterogeneity (I2  = 89.7%); subgroup analysis on venous thrombosis only changed neither effect size (p < 0.0001) nor heterogeneity (I2  = 72.7%). BD participants with ocular involvement had higher HC than those without (p < 0.0001) with moderate heterogeneity (I2  = 40.3%). Conclusion Although causality cannot be inferred, the consistency of the elevation of plasma HC in BD, particularly in patients with active disease, with vascular and ocular involvement suggests an intrinsic involvement of HC in these clinical manifestations.

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.

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