Behcet’s Syndrome

Division of Rheumatology, Duke University Medical Center, Durham, NC, USA.
Drugs (Impact Factor: 4.34). 11/2011; 72(17). DOI: 10.2165/11641370-000000000-00000


Behcet’s syndrome (BS) is a vasculitis, seen more commonly around the Mediterranean and the Far East, and manifests with oral and genital ulcerations, skin lesions, uveitis, and vascular, central nervous system and gastrointestinal involvement. Its natural history of getting less severe over time, more severe disease in males and lack of specific diagnostic testing separates it from other commonly seen conditions in rheumatology. Most of the serious manifestations respond well to immunosuppression, and these are the mainstays of treatment for BS.
BS is more prevalent in regions along the Silk Road, from the Mediterranean to the Far East. The genetic risk factor most strongly associated with BS is the human leukocyte antigen (HLA)-B51 allele. While genetic factors seem to play a role in the development of certain features of BS, there is general consensus that as yet unidentified environmental stimuli are necessary for initiation of disease. Proposed exogenous triggers include both bacterial and viral infections, which may then lead to dysregulation of the immune system, ultimately leading to the phenotypic expression of disease.
The clinical manifestations of BS are protean in nature. While most patients develop mucocutaneous and genital ulcers along with eye disease, other patients may also present with arthritis, frank vasculitis, thrombophlebitis and CNS disease. Interestingly, the manifestations of this illness vary considerably based on gender and ethnicity.
As the phenotypic expression among patients with BS is quite heterogeneous, pharmacological therapy is variable and dependent upon the severity of the disease as well as organ involvement. Treatment for BS overlaps considerably with therapies for other autoimmune diseases, including systemic lupus erythematosus, rheumatoid arthritis and the vasculitides. Pharmacological agents utilized for treatment of BS include corticosteroids, colchicine, azathioprine, and tumour necrosis factor (TNF)-α inhibitors, among others. In this article, we review the salient clinical studies for each drug class along with important side effects as well as drug toxicity monitoring.
Management of the patient with BS is complex and oftentimes requires a multidisciplinary approach. We discuss strategies to assess and stratify patients based on clinical manifestations and disease severity. A summary of drug toxicities as they relate to the aforementioned pharmacological agents, as well as guidelines regarding vaccinations in this patient population, are offered. Finally, we conclude with treatment strategies for the common manifestations of BS along with a discussion of the management of thrombotic disease in these patients.

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    • "The diagnosis of BD is based on clinical criteria and no pathognomonic laboratory findings exist [2]. Proposed exogenous triggers include both bacterial and viral infections, which may then lead to dysregulation of the immune system, ultimately leading to the phenotypic expression of disease [3]. Behc¸et's disease can involve almost every organ and system in the body and has extremely diverse manifestations [4]. "
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    ABSTRACT: Behçet’s disease (BD) is a chronic multisystem disorder characterized by recurrent uveitis, oral aphthae, genital ulcers, and skin lesions. Evidence suggests an exaggerated response to increased cytokine and chemokine production and function. Aim of the work To determine serum tumor necrosis factor-alpha (TNF-α) levels in BD and its relation to clinical manifestations, disease activity and acute phase reactants. Patients and methods Thirty Egyptian male patients with BD (active n = 20, inactive n = 10) and 20 age and sex matched healthy control subjects were included in this study. Serum TNF-α level was determined and correlated with high sensitivity C-reactive protein (hs-CRP), erythrocyte sedimentation rate (ESR) and Behçet’s disease current activity form (BDCAF) Results The TNF-α level of BD patients was significantly higher (30.75 ± 16.94 pg/ml) compared with the control group (5.08 ± 3.19 pg/ml; P < 0.0001). Patients with ocular manifestation had a statistically significant higher TNF-α than those without (P = 0.007). The TNF-α level was significantly higher in those with active disease (36.51 ± 16.36 pg/ml) compared to those inactive (19.24 ± 11.78 pg/ml). However there was a statistically significant difference in ESR and Hs-CRP only between the active group and controls (P = 0.0001 and 0.001, respectively) and not between active and inactive groups. Conclusion High levels of TNF-α indicate the activation of immune response in BD. Serum TNF-α seems to be related to disease activity. High levels of TNF-α were present in patients with active ocular disease, raising the hope of the efficacy of anti TNF-α agents in the treatment of refractory ocular involvement.
    Full-text · Article · Jul 2014 · Egyptian Rheumatologist
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    • "Thus, therapy is mainly based on the type and severity of clinical manifestations and disease duration, as well as number of flares [13]. The mainstay of therapy of isolated aphthosis and acne-like lesions is centred on topical measures [14]. Colchicine at a daily dosage of 1- 2 mg/day can be introduced as an additional option in the management of mucocutaneous signs, as its efficacy has been demonstrated in genital aphthosis and erythema nodosum, as well as in joint involvement displayed by female patients [15] [16]. "
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    ABSTRACT: Behçet's disease (BD) is universally recognized as a multisystemic inflammatory disease of unknown etiology with chronic course and unpredictable exacerbations: its clinical spectrum varies from pure vasculitic manifestations with thrombotic complications to protean inflammatory involvement of multiple organs and tissues. Treatment has been revolutionized by the progressed knowledge in the pathogenetic mechanisms of BD, involving dysfunction and oversecretion of multiple proinflammatory molecules, chiefly tumor necrosis factor- (TNF-) α, interleukin- (IL-) 1β, and IL-6. However, although biological treatment with anti-TNF-α agents has been largely demonstrated to be effective in BD, not all patients are definite responders, and this beneficial response might drop off over time. Therefore, additional therapies for a subset of refractory patients with BD are inevitably needed. Different agents targeting various cytokines and their receptors or cell surface molecules have been studied: the IL-1 receptor has been targeted by anakinra, the IL-1 by canakinumab and gevokizumab, the IL-6 receptor by tocilizumab, the IL12/23 receptor by ustekinumab, and the B-lymphocyte antigen CD-20 by rituximab. The aim of this review is to summarize all current experiences and the most recent evidence regarding these novel approaches with biological drugs other than TNF-α blockers in BD, providing a valuable addition to the actually available therapeutic armamentarium.
    Full-text · Article · Jun 2014 · Mediators of Inflammation
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    • "BD is more prevalent in regions along the silk Road, from the Mediterranean to the far East.30 Approximately 12.5% of uveitis cases from a Saudi Arabian university center were due to BD.24 Uveitis in BD is always non-granulomatous and eventually involves both the anterior and posterior segment of the eye bilaterally. "
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    ABSTRACT: Uveitis is a common cause of preventable blindness although it is consider a sight-threatening condition particularly in cases with posterior segment inflammation. To deal with emergency conditions in uveitis, we must aware of the essential signs and symptoms that reflect a true uveitic emergency. Failure to recognize these essential signs and symptoms of a true uveitic emergency may result in a devastating visual outcome. This review provides general ophthalmologists and residents, clinical guidelines for the main uveitic entities that require immediate recognition and urgent intervention in the emergency room to prevent severe permanent visual loss.
    Full-text · Article · Mar 2014 · Middle East African journal of ophthalmology
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