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Behcet's disease physiopathology: a contemporary review.

ABSTRACT: Behçet's disease, also known as the Silk Road Disease, is a rare systemic vasculitis disorder of unknown etiology. Recurrent attacks of acute inflammation characterize Behçet's disease. Frequent oral aphthous ulcers, genital ulcers, skin lesions and ocular lesions are the most common manifestations. Inflammation is typically self-limiting in time and relapsing episodes of clinical manifestations represent a hallmark of Behçet's disease. Other less frequent yet severe manifestations that have a major prognostic impact involve the eyes, the central nervous system, the main large vessels and the gastrointestinal tract. Behçet's disease has a heterogeneous onset and is associated with significant morbidity and premature mortality. This study presents a current immunological review of the disease and provides a synopsis of clinical aspects and treatment options.

SUBMITTER: Zeidan MJ

PROVIDER: S-EPMC4751097 | biostudies-literature | 2016 Dec

REPOSITORIES: biostudies-literature

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Behçet's disease physiopathology: a contemporary review.

Zeidan Mohamad J MJ Saadoun David D Garrido Marlene M Klatzmann David D Six Adrien A Cacoub Patrice P

Auto- immunity highlights 20160212 1

Behçet's disease, also known as the Silk Road Disease, is a rare systemic vasculitis disorder of unknown etiology. Recurrent attacks of acute inflammation characterize Behçet's disease. Frequent oral aphthous ulcers, genital ulcers, skin lesions and ocular lesions are the most common manifestations. Inflammation is typically self-limiting in time and relapsing episodes of clinical manifestations represent a hallmark of Behçet's disease. Other less frequent yet severe manifestations that have a m ...[more]

PMID: 26868128

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The immunogenetics of Behcet's disease: A comprehensive review.

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Project description:Behcet's disease (BD) is a chronic systemic inflammatory vasculitis of unknown etiology characterized by recurrent episodes of oral aphthous ulcers, genital ulcers, skin lesions, ocular lesions, and other manifestations. Although the pathogenesis of BD is unclear, some studies have shown that immunological aberrations play an important role in the development and progression of BD. Infection-related trigger factors, including antigens and autoantigens, are believed to mediate the development of BD in patients with a genetic predisposition and subsequently activate the innate and adaptive immune systems, resulting in the production of numerous cytokines and chemokines to combat the infection-related factors. The study of the immunological mechanism of BD paves the way for the development of innovative therapies. Recently, novel biotherapy approaches, including interferon-α (IFN-α), tumor necrosis factor-α (TNF-α) antagonists, and other agents that target interleukins and their receptors, have shown promising results in the treatment of patients with refractory BD and have improved the prognosis of BD. In this review, we provide the current concepts of BD immunopathogenesis.

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Genetics of Behcet's Disease.

Project description:Behçet's disease (BD) is a systemic inflammatory disorder characterized mainly by recurrent oral and genital ulcers and eye involvement. Although the pathogenesis remains poorly understood, a variety of studies have demonstrated that genetic predisposition is a major factor in disease susceptibility. Peculiar geographical distribution of BD along the ancient Silk Road has been regarded as evidence supporting genetic influence. The observed aggregation of BD in families of patients with BD is also supportive for a genetic component in its etiology. HLA-B51 (B510101 subtype) is the most strongly associated genetic marker for BD in countries on the Silk Road. In recent years, several genome-wide association studies and genetic polymorphism studies have also found new genetic associations with BD, which may have a supplementary role in disease susceptibility and/or severity. The author reviewed the HLA and non-HLA genetic association studies.

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Project description:Background and objectivesBehçet's disease (BD) is a systemic vasculitis that is characterized by genital, oral, or skin lesions, uveitis, and vascular complications. Studies have shown that increased arterial stiffness is common in systemic immune and inflammatory diseases such as rheumatoid arthritis and systemic lupus erythematosus. However, current research has not yet determined whether patients with BD have increased arterial stiffness. This meta-analysis compares arterial stiffness parameters in subjects with a BD diagnosis to normal subjects.Subjects and methodsA comprehensive search of the MEDLINE and EMBASE databases was performed from the database beginning through May 2016. Observation studies were included in this analysis if they assessed the association between BD and arterial stiffness in adult subjects. BD patients met the International Study Group criteria for a diagnosis of Behçet's disease. Aortic stiffness was assessed using carotid-femoral pulse wave velocity (PWV) measurements as an indicator. Pooled mean difference (MD) of PWV and 95% confidence intervals (CI) were calculated using a random-effect, generic inverse variance meta-analysis. The between-study heterogeneity of effect-size was quantified using the Q statistic and I2.ResultsData were extracted from four observational studies that included 303 subjects. PWV is significantly higher in patients with Behçet's disease compared with controls (MD=0.74;95%, CI: 0.28-1.20, p=0.002, I2=63%).ConclusionIn this meta-analysis, we observed that PWV, an ideal indicator of arterial stiffness, is increased in patients with Behçet's disease compared with the controls. Prospective studies in a large population should be done to determine the pathophysiological and prognostic implications of increased arterial stiffness in BD.

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Behcet's Disease: Is There Geographical Variation? A Review Far from the Silk Road.

Project description:Behcet's Disease (BD) is a systemic vasculitis characterized by the triad of recurrent mouth and genital ulcers with eye involvement. To date there are no laboratory tests specific for the disease and diagnosis continues to remain on clinical grounds. Multiple criteria have been created as guides for diagnosis; however, given the wide spectrum of organ involvement, some cases remain undiagnosed. The diagnosis of Behcet's Disease may only be made over time as the clinical manifestations emerge sometimes separated by months and even years. With an increased recognition of this disease it has become apparent that there is geographical variation in clinical manifestations. In particular cardiac manifestations are not seen commonly in Caucasians compared to Asian and Middle Eastern patients, while neurological manifestations are more common in Caucasians. Use of immunosuppressive and immunomodulatory drugs to suppress inflammation remains the cornerstone of treatment.

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