Behcet’s Syndrome

Division of Rheumatology, Duke University Medical Center, Durham, NC, USA.
Drugs (Impact Factor: 4.13). 72(17). DOI: 10.2165/11641370-000000000-00000

ABSTRACT 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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    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.
    Mediators of Inflammation 06/2014; 2014:107421. DOI:10.1155/2014/107421 · 2.42 Impact Factor
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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.
    Middle East African journal of ophthalmology 03/2014; 21(3):251-258. DOI:10.4103/0974-9233.134687
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    ABSTRACT: PurposeTo describe the echocardiographic findings detected as first manifestations of Behçet's disease (BD) and compare these findings with those detected in established cases of BD receiving regular medical treatment.Methods Two groups of patients were studied. Group 1 comprised 41 patients with BD on regular medical treatment. Group 2 comprised 5 previously healthy patients who presented to the cardiology department because of cardiac symptoms and were diagnosed in retrospect during hospitalization as BD. Thirty-two age- and sex-matched individuals served as control subjects (control group). All patients and controls underwent complete M-mode, two-dimensional, and Doppler transthoracic echocardiographic examinations.ResultsOf the 41 treated patients with BD (mean age: 32 ± 8 years, 90% males, mean duration since diagnosis: 14.5 years), only 1 patient was found to have severe aortic regurgitation secondary to aortic root dilation. On the other hand, the 5 previously healthy patients who presented with cardiac symptoms (mean age: 24 ± 6 years, all males) had significant cardiac involvement and evident echocardiographic findings (P < 0.001). Four cases had intracardiac masses: 3 in the right atrium (RA), 1 in the right ventricle (RV), while the last patient had pericardial effusion (PE). All these patients were diagnosed in retrospect as BD. The RA masses disappeared on medical therapy, while the RV mass was surgically excised and proved to be multiple thrombi histopathologically. The patient with PE had recurrent attacks of massive effusion so a pericardial window was performed surgically.Conclusion Diagnosis of BD might be initially suspected by the cardiologists based on certain echocardiographic findings, namely the presence of right-sided masses. Diagnosis of BD in such patients has important therapeutic implications and accordingly prognostic value.
    Echocardiography 09/2014; 31(8). DOI:10.1111/echo.12601 · 1.25 Impact Factor