scholarly journals New Evidence-Based Treatment Approach in Behçet's Disease

2012 ◽  
Vol 2012 ◽  
pp. 1-11 ◽  
Author(s):  
Erkan Alpsoy
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Age Of Onset ◽  
Behçet's Disease ◽  
Systemic Vasculitis ◽  
Severe Disease ◽  
Immunosuppressive Agents ◽  
Evidence Based ◽  
Close Monitoring ◽  
Behcet's Disease

Behçet's disease (BD) is a chronic, relapsing, and debilitating systemic vasculitis of unknown aetiology with the clinical features of mucocutaneous lesions, ocular, vascular, articular, neurologic, gastrointestinal, urogenital, and pulmonary involvement. The disease is much more frequent along the ancient “Silk Route” extending from Eastern Asia to the Mediterranean basin, compared with Western countries. The disease usually starts around the third or fourth decade of life. Male sex and a younger age of onset are associated with more severe disease. Although the treatment has become much more effective in recent years, BD is still associated with severe morbidity and considerable mortality. The main aim of the treatment should be the prevention of irreversible organ damage. Therefore, close monitoring, early, and appropriate treatment is mandatory to reduce morbidity and mortality. The treatment is mainly based on the suppression of inflammatory attacks of the disease using immunomodulatory and immunosuppressive agents. In this paper, current state of knowledge regarding the therapeutic approaches is outlined. To provide a rational framework for selecting the appropriate therapy along the various treatment choices, a stepwise, symptom-based, evidence-based algorithmic approach was developed.

scholarly journals Behcet’s disease: a review

2021 ◽  
Author(s):  
Daniela Ann Reyes-Weaver ◽  
Kevin Luis Plata-Jimenez ◽  
Raul Melo-Acevedo
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Age Of Onset ◽  
Behçet's Disease ◽  
Systemic Vasculitis ◽  
Skin Lesions ◽  
Vascular Lesions ◽  
Severe Condition ◽  
Behcet's Disease ◽  
Oral Ulcers

Behcet's disease (BD) is a complex systemic vasculitis with an etiopathogenesis that remains unclear. It has a strong geographic association as well as a genetic propensity linked to the HLA-B51 factor and interactions between genetic and environmental factors. The typical age of onset is 25 to 40 years old and it is more common in men, who also have a more severe condition. Occlusive vasculitis is the hallmark of this condition, which can affect vessels of all diameters. Oral ulcers, genital ulcers, skin lesions, pathergy reaction as well as involvement of other systems and organs such as ophthalmic, neurological and vascular lesions, among others, are used to make the diagnosis. Uncontrolled neutrophil activation, activation of the humoral and cell immune systems, toxic proteins and infectious agents such as herpes simplex and streptococci are all involved in their pathophysiology. Due to the heterogeneity and several systems affected, the treatment is individualized and focused on treating each clinical manifestation.

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 case of intestinal Behcet’s disease under Adalimumab

2017 ◽  
Vol 4 (3) ◽  
pp. 62
Author(s):  
David T. Dulaney ◽  
Wassem Juakiem ◽  
Katherine Cebe ◽  
Angelo H. Paredes
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Immunosuppressive Agents ◽  
African American Female ◽  
Behcet's Disease ◽  
Oral Ulcers ◽  
Adalimumab Therapy ◽  
Tnfα Inhibitors ◽  
Tnfα Inhibitor

Behcet’s disease (BD) is a multisystem mucocutaneous inflammatory condition characterized by recurrent genital and oral ulcers, ocular inflammation, and can involve the gastrointestinal tract. Treatment involves the usage of immunosuppressive agents to control the disease with glucocorticoids utilized for treatment of flares. Tumor necrosis factor inhibitors are frequently used to control the disease as well. We present the case of a 40 years old African American female presenting with intestinal BD that was refractory to adalimumab therapy. In conjunction with glucocorticoids, the patient’s intestinal disease was controlled with infliximab therapy. Currently, there have been no studies comparing the efficacy of TNFα inhibitors on the treatment of BD. Future studies are needed to compare the efficacy of TNFα inhibitor agents in the treatment of intestinal manifestations of BD.

scholarly journals Evidence-based practice in Behçet’s disease: identifying areas of unmet need for 2014

2014 ◽  
Vol 9 (1) ◽  
pp. 16 ◽  
Author(s):  
Robert J Barry ◽  
Bharat Markandey ◽  
Rahul Malhotra ◽  
Henry Knott ◽  
Nikita Joji ◽  
...  
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Evidence Based Practice ◽  
Behçet's Disease ◽  
Unmet Need ◽  
Evidence Based ◽  
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 Effectiveness and Safety of Chinese Medicine Decoctions for Behcet’s Disease: A Systematic Review and Meta-Analysis

2021 ◽  
Vol 2021 ◽  
pp. 1-19
Author(s):  
Jingxian Yan ◽  
Yi Yan ◽  
Andrew Young ◽  
Zhiyong Yan ◽  
Zhimin Yan
Keyword(s):  
Chinese Medicine ◽  
Adverse Events ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Systemic Vasculitis ◽  
Meta Analysis ◽  
Clinical Feature ◽  
Behcet's Disease ◽  
Secondary Outcomes

Background. Behcet’s disease (BD) is an autoimmune disease of systemic vasculitis with an unclear pathogenesis. Although western medicines remain the mainstay interventions, effectiveness and safety are significant challenges. Complementary and alternative medicine, including herbal medicine, are gaining more attention. Chinese medicine decoctions, which have been used for centuries, are the most common form of traditional therapies. Objective. The purpose of the review was to evaluate the effectiveness and safety of Chinese medicine decoctions in the treatment of BD. Methods. Randomized controlled trials (RCTs) for BD treatment with Chinese medicine decoctions were searched in six electronic databases until March 2021. Primary outcomes were total effective rate, recovery rate, and recurrence rate. Secondary outcomes were clinical feature scores (oral ulcers, eye lesions, genital ulcers, skin lesions, arthropathies, fever, and pathergy reactions) and laboratory index levels (erythrocyte sedimentation rate, C-reactive protein, and immunoglobulin A). The risk of bias was assessed with the Cochrane Handbook, and a meta-analysis was performed with RevMan 5.4.1. Results. Sixteen RCTs with 924 patients were included in the review. The meta-analysis indicated that Chinese medicine decoctions were effective for BD when compared with control groups for all the primary outcomes and 7/10 of the secondary outcomes. Adverse events were reported in 11 of the 16 RCTs, with the Chinese medicine decoctions possibly having fewer adverse events than western drugs. This review included a range of classical prescriptions. An additional meta-analysis of modified Gancao Xiexin Decoction for BD treatment was conducted. Gancao Xiexin decoction is also discussed as a representative prescription, as well as high-frequency herbs, and warrants further exploration for individualized medicine and pharmacology. Conclusion. Chinese medicine decoctions have the potential to be effective and safe for treating BD. However, additional well-designed RCTs are needed to confirm the findings because of the unsatisfactory quality of the included studies.

scholarly journals Isomer-Specific Monitoring of Sialylated N-Glycans Reveals Association of α2,3-Linked Sialic Acid Epitope With Behcet’s Disease

2021 ◽  
Vol 8 ◽  
Author(s):  
Nari Seo ◽  
Hyunjun Lee ◽  
Myung Jin Oh ◽  
Ga Hyeon Kim ◽  
Sang Gil Lee ◽  
...  
Keyword(s):  
Sialic Acid ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Systemic Vasculitis ◽  
Multiple Reaction Monitoring ◽  
High Sensitivity ◽  
High Specificity ◽  
Unknown Etiology ◽  
Behcet's Disease

Behcet’s disease (BD) is an immune disease characterized by chronic and relapsing systemic vasculitis of unknown etiology, which can lead to blindness and even death. Despite continuous efforts to discover biomarkers for accurate and rapid diagnosis and optimal treatment of BD, there is still no signature marker with high sensitivity and high specificity. As the link between glycosylation and the immune system has been revealed, research on the immunological function of glycans is being actively conducted. In particular, sialic acids at the terminus of glycoconjugates are directly implicated in immune responses, cell–cell/pathogen interactions, and tumor progression. Therefore, changes in sialic acid epitope in the human body are spotlighted as a new indicator to monitor the onset and progression of immune diseases. Here, we performed global profiling of N-glycan compositions derived from the sera of 47 healthy donors and 47 BD patients using matrix-assisted laser desorption/ionization-time of flight mass spectrometry (MALDI-TOF MS) to preferentially determine BD target glycans. Then, three sialylated biantennary N-glycans were further subjected to the separation of linkage isomers and quantification using porous graphitized carbon-liquid chromatography (PGC-LC)/multiple reaction monitoring (MRM)-MS. We were able to successfully identify 11 isomers with sialic acid epitopes from the three glycan compositions consisting of Hex5HexNAc4NeuAc1, Hex5HexNAc4Fuc1NeuAc1, and Hex5HexNAc4NeuAc2. Among them, three isomers almost completely distinguished BD from control with high sensitivity and specificity with an area under the curve (AUC) of 0.945, suggesting the potential as novel BD biomarkers. In particular, it was confirmed that α2,3-sialic acid at the terminus of biantennary N-glycan was the epitope associated with BD. In this study, we present a novel approach to elucidating the association between BD and glycosylation by tracing isomeric structures containing sialic acid epitopes. Isomer-specific glycan profiling is suitable for analysis of large clinical cohorts and may facilitate the introduction of diagnostic assays for other immune diseases.

scholarly journals Pediatric Behcet's Disease Patient with Cardiac Valvular Lesions: case report and a review of the literature

2021 ◽  
Author(s):  
Xin Yao ◽  
Xin-Ning Wang ◽  
Zhi-Xuan Zhou ◽  
Sheng-Nan Li ◽  
Jianming Lai
Keyword(s):  
Surgical Treatment ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Cardiac Involvement ◽  
Behçet's Disease ◽  
Disease Patient ◽  
Close Monitoring ◽  
Primary Therapy ◽  
Behcet's Disease ◽  
Valvular Lesions

Abstract Background To explore the clinical characteristics, treatment regimen, and prognosis of cardiac valvular involvement in pediatric Behcet's disease. Methods Retrospective analysis was conducted on the clinical data obtained from one pediatric patient suffering from Behcet's disease with cardiac valvular involvement. Present study also reviewed the literature related to cardiac involvement in pediatric Behcet's disease. Results The disease onset in the female patient was reported at the age of 3 years, with clinical symptoms, including apthous ulcers, fever, perianal ulcers, and erythema nodosum, accompanied by a significant increase in the inflammatory markers. Echocardiography indicated that aortic valve (AV) lesions, which were absent initially, developed later, gradually exacerbated. Initially, she was diagnosed as "infectious disease" and "systemic inflammatory response syndrome", and antibiotic therapy proved ineffective. After an accurate diagnosis of Behcet's disease, the patient was given glucocorticoids, immunosuppressants, and biologics, which resulted in the relief of the symptoms; however, cardiac valvular lesions continued to exacerbate. Subsequent treatment included medical treatment and surgical treatment, and the patient's condition was stable at follow-up. Conclusions Cardiac valvular lesions in pediatric Behcet's disease are rare with insidious onset, enhancing the propensity of misdiagnosis or missed diagnosis. Main manifestations include recurrent oral ulceration, skin lesions, and fever. Cardiac involvement may occur early, yet the symptoms are insidious, requiring comprehensive physical examination and close monitoring of echocardiography. Glucocorticoids combined with immunosuppressants are the primary therapy for cardiac involvement in Behcet's disease. When cardiac valvular damage and dysfunction are observed, treatment regimens can be jointly recommended by multidisciplinary consultation to conduct the surgical treatment on time.

scholarly journals COVID-19 ON BEHÇET’S DISEASE PATIENT : SHED LIGHT ON SHADOWS (CASE REPORT)

2020 ◽  
Vol 8 (11) ◽  
pp. 49-52
Author(s):  
Hanane Ezzouine ◽  
Mehdi Simou ◽  
Aymane El Louadghiri ◽  
Mounir Louardi ◽  
Imane Khaoury ◽  
...  
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Systemic Vasculitis ◽  
Disease Patient ◽  
Family Type ◽  
Behcet's Disease ◽  
New Family ◽  
Arterial Involvement ◽  
Shed Light

A new family type of coronavirus (SARS-CoV-2) was first seen in Wuhan, China name coronavirus disease 2019 (COVID-19). COVID-19 primarily attacks the respiratory system, but several studies have shown that infection with SARS-COV-2 can cause thrombosis and have therefore considered COVID-19 to be a prothrombotic disease. Behçet's disease (MB) is a systemic vasculitis. Its ethiopathogeny is still poorly understood. Blood vessels of any caliber can be affected. Arterial involvement, such as thrombosis and / or aneurysm, is possible. This article reports the case of SARS-COV-2 infection in a patient with behcet's disease.

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