scholarly journals Treatment of Behçet's Disease: An Algorithmic Multidisciplinary Approach

2021 ◽  
Vol 8 ◽  
Author(s):  
Erkan Alpsoy ◽  
Pietro Leccese ◽  
Giacomo Emmi ◽  
Shigeaki Ohno
Keyword(s):  
Behçet’S Disease ◽  
Multidisciplinary Approach ◽  
Behcet’S Disease ◽  
Cardiac Involvement ◽  
Age Of Onset ◽  
Behçet's Disease ◽  
Immunosuppressive Drugs ◽  
Unknown Etiology ◽  
Multisystem Disease ◽  
Behcet's Disease

Behçet's disease (BD) is a chronic, relapsing inflammatory, multisystem disease of unknown etiology. The disease has a wide clinical spectrum of mucocutaneous lesions and ocular, vascular, articular, neurologic, gastrointestinal and cardiac involvement. Although the number of effective drugs used in the disease's treatment has increased in recent years, BD is still associated with severe morbidity because of mainly mucocutaneous, articular and ocular symptoms and an increased mortality because of large vessel, neurological, gastrointestinal and cardiac involvement. Many factors are associated with a more serious course, such as male gender and a younger age of onset. While the severity of the disease is more pronounced in the first years of the disease, it decreases in most patients after the age of forties. The primary goal of treatment should be the prevention of irreversible organ damage. Therefore, early diagnosis and appropriate treatment and close follow-up are mandatory to reduce the morbidity and mortality of the disease. Treatment varies depending on the organ involved and the severity of the involvement. For all these reasons, the treatment should be personalized and arranged with a multidisciplinary approach according to the organs involved. Treatment is mainly based on suppression of the inflammatory attacks of the disease using local and systemic immunomodulatory and immunosuppressive drugs. In this review, based on the mainly controlled studies and personal experience in clinical practice and basic research in this field, we propose a stepwise, symptom-based, algorithmic approach for the management of BD with a holistic perspective.

scholarly journals Cardiac involvement in Behçet's disease

1985 ◽  
Vol 28 (3) ◽  
pp. 345-348 ◽  
Author(s):  
Carolyn A. Bowles ◽  
Audrey M. Nelson ◽  
Stephen C. Hammill ◽  
J. Desmond O'duffy
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Cardiac Involvement ◽  
Behçet's Disease ◽  
Behcet's Disease

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.

scholarly journals Pulmonary embolism in Behcet’s disease: a case report

2020 ◽  
Vol 90 (4) ◽  
Author(s):  
Despoina Moumtzi ◽  
Marianna Kakoura
Keyword(s):  
Pulmonary Embolism ◽  
Differential Diagnosis ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Rare Complication ◽  
Pulmonary Involvement ◽  
Unknown Etiology ◽  
Disease Exacerbation ◽  
Behcet's Disease

Behcet’s disease (BD) is a vasculitis of unknown etiology. It is often correlated with thrombophilic factors such as V Leiden. Pulmonary involvement is reported in 1-10% of patients. The most common manifestations are pulmonary aneurysms while pulmonary embolism is a rare complication. A 41-year old man with BD and V Leiden heterozygosity complained of pleurodynia and fever. Pleurodynia deteriorated in the following days and PE was confirmed by CT angiography, without the presence of aneurysms. After the exclusion of the antiphospholipid syndrome, a therapeutic dose of apixaban was initiated. Two weeks later, pleurodynia relapsed in combination with pleural effusion unilaterally. These findings were attributed to disease exacerbation. For this reason, we decided to enhance the immunosuppressive therapy. Six months later, CTPA showed complete remission of the clots. Vasculitis predisposes to thrombosis with or without coexisting thrombophilia. Clinicians should include them in their differential diagnosis and provide personalized treatment, based on immunosuppressants.

scholarly journals Behcet’s Disease with Intracardiac Thrombus Presenting with Fever of Unknown Etiology

2015 ◽  
Vol 2015 ◽  
pp. 1-3
Author(s):  
Sajal Ajmani ◽  
Durga Prasanna Misra ◽  
Deep Chandh Raja ◽  
Namita Mohindra ◽  
Vikas Agarwal
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Thrombus Formation ◽  
Young Male ◽  
Unknown Etiology ◽  
High Dose ◽  
Intracardiac Thrombus ◽  
High Grade Fever ◽  
Behcet's Disease

A young male was referred to us for evaluation of fever of unknown origin (FUO). He had history of recurrent painful oral ulcers for one year and moderate to high grade fever, pustulopapular rash, and recurrent genital ulcers for 6 months and hemoptysis for 3 days. He was detected to have intracardiac thrombi and pulmonary arterial thrombosis along with underlying Behcet’s disease (BD). Patient responded to high dose prednisolone (1 mg/Kg/day) along with monthly parenteral cyclophosphamide therapy. This case highlights the fact that BD is an important cause for pulmonary artery vasculitis with intracardiac thrombus formation, and such patients can present with FUO.

scholarly journals 0274: Cardiac involvement in Behcet’s disease

2015 ◽  
Vol 7 (1) ◽  
pp. 47
Author(s):  
Tounsi Ahmed ◽  
Abid Leila ◽  
Frikha Feten ◽  
Akrout Malek ◽  
Elaoud Sahar ◽  
...  
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Cardiac Involvement ◽  
Behçet's Disease ◽  
Behcet's Disease

scholarly journals Aortic thrombosis in a patient with a late diagnosis of Behcet's disease

2015 ◽  
Vol 14 (2) ◽  
pp. 193-196
Author(s):  
Ana Bittencourt Detanico ◽  
Marcelo Luiz Brandão ◽  
Ly de Freitas Fernandes ◽  
Carolina Parreira Ribeiro Camelo ◽  
Juliano Ricardo Santana dos Santos
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Systemic Vasculitis ◽  
Thromboangiitis Obliterans ◽  
Unknown Etiology ◽  
Behcet's Disease ◽  
Oral Ulcers ◽  
Arterial Involvement ◽  
Genital Ulcers

Behcet's disease is form of systemic vasculitis of unknown etiology. One surprising feature is that arterial involvement is less common in this disease than venous forms, accounting for 1 to 7% of patients. In 7 to 30% of cases the vascular symptoms precede the clinical diagnosis of Behcet. We describe the case of a patient with Thromboangiitis Obliterans who was treated with lumbar sympathectomy and an aortobiiliac bypass. Around 1 year later he was diagnosed with Behcet's disease after presenting with oral ulcers, genital ulcers and scaling lesions on the hands.

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.

scholarly journals Successful Treatment of Intracardiac and Pulmonary Thrombi in Behçet’s Disease with Oral Anticoagulant and Immunosuppressive Therapy

2012 ◽  
Vol 55 (4) ◽  
pp. 186-188 ◽  
Author(s):  
Uğur Canpolat ◽  
Hikmet Yorgun ◽  
Ali Akdoğan ◽  
Kudret Aytemir
Keyword(s):  
Immunosuppressive Therapy ◽  
Behçet’S Disease ◽  
Oral Anticoagulant ◽  
Behcet’S Disease ◽  
Cardiac Involvement ◽  
Behçet's Disease ◽  
Chronic Inflammatory Disease ◽  
Vascular Involvement ◽  
Behcet's Disease ◽  
Ventricular Thrombus

Behçet’s disease is a multisystem chronic inflammatory disease generally presenting as recurrent oral-genital ulcers and uveitis. Vascular involvement is a common presentation. However, cardiac involvement is rare in this disorder. In this case report, we present a large right ventricular thrombus detected both in computed tomography and echocardiography in a 32 year-old male patient with complaints of fever, haemoptysis and weight loss. Intracardiac thrombus showed both clinical and radiological regression with oral anticoagulant and immunosuppressive therapy.

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