scholarly journals Efficacy of TNF-α antagonist and other immunomodulators in the treatment of patients with ophthalmologic manifestations of Behcet's disease and HLA B51 positive vasculitis

2012 ◽  
Vol 69 (2) ◽  
pp. 168-174 ◽  
Author(s):  
Gordana Zlatanovic ◽  
Svetlana Jovanovic ◽  
Dragan Veselinovic ◽  
Maja Zivkovic
Keyword(s):  
Visual Acuity ◽  
Disease Activity ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Unknown Etiology ◽  
Behcet's Disease ◽  
Therapeutic Method ◽  
Tnf Antagonist ◽  
Pathergy Test

Bacground/Aim. Behcet's disease is genetically conditioned, immune-mediated multisystem occlusive vasculitis of small blood vessels, espesially venules, of unknown etiology. The aim of this study was to analyze the clinical features, disease activity and therapy of the patients with ophthalmologic manifestation of Behcet's disease. Methods. In this study symptoms and signs of the disease were analyzed both prospectively and retrospectively during the active manifestation of the disease. The diagnosis was reached according to the International Criteria for Behcet's Disease (2006). The treatment effects were evaluated based on the presence of the best corrected visual acuity and the inflammation of the vitreous humour before and after the application of our therapeutic method. The applied therapeutic modality consisted of the primary application of corticosteroid therapy in the active stage of the disease complemented with the choice of drugs from the immunosuppressive group. In this study there drugs were cyclosporine or methotrexate. A treatment refractory patients with poor vision prognosis were treated with a third drug, the biological preparation infliximab, a tumor necrosis factor-alpha (TNF-?) antagonist. Results. The mean age of 11 patients with ophthalmologic manifestation of Behcet's disease was 50.6 years. HLA B-5(51) was positive in 81% of the patients while 36% of the patients had positive pathergy test. Changes in affected eyes included vitritis (100%), posterior uveitis (45%), panuveitis (54%), retinal vasculitis (54%), cystoid macular edema (54%), and cystoid degeneration (18%). Increased intraocular pressure was observed in 27% of the patients. There was no statistically significant variation in disease activity parameters in any of the patients (p > 0.05). A statistically significant improvement in visual acuity (p < 0.05) and a high statistically significant decrease of inflammation of the worst affected eyes (p = 0.001) were detected. Conclusion. Our therapeutic method is useful for producing the optimal therapeutic plan for the acute - chronic stage of the difficult ophtamological manifestation of Behcet's desease as well as the prevention of relapse. However the high cost of the therapy and the potential complications should be taken into consideration when prescribing this therapy, especially a TNF-? antagonist.

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 AB0305 IS THERE HYPERCOAGULATION IN PATIENTS WITH ANTIPHOSPHOLIPID SYNDROME AND BEHCET’S DISEASE?

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1178.2-1179
Author(s):  
N. Seredavkina ◽  
T. Reshetnyak ◽  
T. Lisitsyna ◽  
A. Lila
Keyword(s):  
Disease Activity ◽  
Behçet’S Disease ◽  
Antiphospholipid Syndrome ◽  
Anticoagulant Therapy ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Unknown Etiology ◽  
Thrombin Time ◽  
Behcet's Disease ◽  
Coagulation Tests

Background:Whereas antiphospholipid syndrome (APS) is a non-inflammatory vasculopathy and is associated with thrombosis in 98% of cases, Behcet’s disease (BD) is a systemic vasculitis of unknown etiology, characterized by vascular damage of any calibre. Both venous and arterial thromboses occur in 45% of BD patients and are associated not with hypercoagulable disease but with inflammatory changes in the vascular wall mediated by hypersecretion of pro-inflammatory cytokines and endothelial cells dysfunction. Thrombodynamics (TD) is a new global test for diagnosing plasma haemostasis disorders, identifying bleeding and thrombosis risks, and can be used to detect a prothrombotic state and assess the influence of disease activity and course on the hypercoagulation process.Objectives:comparative assessment of TD in patients with APS and BD before anticoagulant therapy (AC).Methods:The study included 20 patients (9 APS and 11 BD) and 8 age and sex-matched healthy controls (HC). None of the subjects received AC. Thromboses in past history were registered in 5/9 (55%) APS patients and 2/11 (18%) BD patients and none of HC. Fetal loss occurred only in women with APS (4/4 (100%) who had pregnancy during the disease). All the patients were hospitalized and underwent fool investigation according to the diagnosis including TD, local coagulation tests and antiphospholipid antibodies profile.Results:the velocity of clot growth in APS was lower, than in BD and in HC: 23.7 [22.6; 24.7] vs 29.0 [28.2; 34.4] and 31.1 [28.9; 33.5] Um/min, respectively (р=0.001). Clot size at 30 minutes in APS also was lower, than in BD and HC: 972.1 [921.3; 1007.4] vs 1152.7 [1098.3; 1225.4] and 1226.6 [1140.5; 1295.1] Um, respectively (р=0.001). Spontaneous clotting was registered only in 2 BD patients in mean time 2 minutes. Clot density and lag time (Tlag, the delay between the test start and the onset of clot formation) were the same in all three groups. Prolonged APTT was found in APS (33.7 [30.6; 47.1] sec) and normal APTT - in BD (30.9 [29.1; 31.1] sec) and HC (29.7 [28.2; 30.8] sec). Increased soluble fibrin-monomer complexes were revealed in all APS patients (100%), 91% BD patients and 25% HC (р=0.01). After interpretation the TD results were distributed as follows: hypocoagulation was noted in 1 APS patient with a positive lupus anticoagulant, while all other APS patients had normocoagulation. Thrombotic readiness status (TRS) was diagnosed only in 2 BD patients. The frequency of normocoagulation and hypercoagulation did not differ between BD and HC. Local coagulation tests (APTT, thrombin time, prothrombin time) were the same depending on hypo- and hypercoagulation by TD results. Fibrinogen level in BD patients with hypercoagulation was higher, than in BD patients with normocoagulation and TRS: 4.2 [3.6; 4.7] vs 2.8 [2.7; 3.0] and 2.8 [2.7;2.8] g/l respectively, р=0.04. BD activity by Behcet’s Disease Current Activity Form correlated with stationary velocity of clot growth (Rs = 0.68, p<0.05).Conclusion:Thrombodynamics results showed: before anticoagulant therapy there were normocoagulation with a tendency to hypocoagulation in APS and hypercoagulation in BD. In BD hypercoagulation associated with disease activity.Disclosure of Interests:None declared

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.

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