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.Electronic supplementary materialThe online version of this article (doi:10.1007/s13317-016-0074-1) contains supplementary material, which is available to authorized users.
Highlights
Behcet’s disease, known as the Silk Road Disease, is a rare systemic vasculitis disorder of unknown etiology
Behcet’s Disease (BD) is a rare systemic vasculitis disorder of unknown etiology characterized by recurrent attacks of oral aphthous ulcers, genital sores, and ocular lesions
Clinical manifestations of acute inflammation are typically self-limiting in time with relapsing episodes of varied intensity leading to sequelae
Summary
Autoimmun Highlights (2016) 7:4 third and the fourth decades of age, infrequently developing before puberty or after the fifth decade of life. The disease appears to occur more frequently and is associated with more severe ocular, neurological, and cardiovascular clinical manifestations in males [1, 3]. Ocular symptoms occur more frequently amongst males and are associated with disease severity, even though prognosis is improving with the use of aggressive immunosuppressant therapy [1, 5] They usually occur 2–3 years after the onset of oral or genital ulcerations but remain the first disease manifestation in 10–20 % of patients [1]. Neurological involvement in BD (neuro-BD) occurs in 5–10 % of patients and is more frequent in males [18, 19] It usually occurs around 5 years after the onset of the disease and is associated with long-term morbidity and mortality [1, 4, 20].
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