Knitting the Threads of Silk through Time: Behçet’s Disease—Past, Present, and Future

Abstract

Behçet's disease (BD) is a chronic relapsing vasculitis that affects vessels of all types and sizes with a broad spectrum of phenotypic heterogeneity and complex immunopathogenesis. Efforts by the scientific community to resolve the unmet needs of BD and gaps in our knowledge have been hampered by considerable challenges that primarily relate to the rare nature of the disease in many parts of the world and its heterogeneity. Controversies remain in many aspects of the disease including the diagnostic criteria, immunopathogenesis and biomarker discovery, geographical variation, and therapeutic considerations. In this review, we highlight recent advances in our scientific understanding of BD, shed new insights into diagnostic and treatment strategies, and discuss residual gaps in our knowledge that will serve as the basis for current and future research.

Behçet’s disease presentations and care outcomes in a tertiary hospital in south‐western Saudi Arabia

“…The prevalence of vascular BD is comparatively lower in Asian countries, including Singapore (5.4%), Hong Kong (11%), Korea (1.8%) indicating higher preponderance (19%‐34%) of vascular BD in Arab populations compared to non‐Arab Asian populations (7%‐12.5%) . Arterial lesions occurring in the form of aneurysms, particularly pulmonary aneurysm although rare, has poor prognosis, leading to mortality . Aneurysms can occur in the main pulmonary arteries or their branches and the probable pathogenesis of aneurysms in BD is anticipated to be the removal of vasa vasorum via inflammatory processes, which impairs nutrient flow to the aortic wall and disrupts media, thereby leading to the development of saccular aneurysmal dilation .…”

“…Behçet's disease (BD) is a rare, chronic, recurrent, multisystem inflammatory disorder of unknown etiology . The disease was named after a Turkish dermatologist Dr Hulushi Behçet who first described the disease in 1937 as a symptomatic triad of relapsing aphthous oral ulcers, genital ulcers, and uveitis in 3 Eastern Mediterranean patients . Despite its worldwide occurrence, BD is more prevalent in countries along the ancient Silk Road extending from the Mediterranean to Middle‐East Asia to Far East Asia .…”

“…Turkey has the highest prevalence of BD, ranging from 20‐602/100 000 population followed by Iran having 68/100 000 population . The prevalence of BD in Saudi Arabia was 20/100 000, in Iraq 17, in Israel 15.2, in Japan 13.5, in France 7.1, in Germany 2.26, in the UK 0.64, and in the USA 5.2 .…”

“…Genital ulcers occur within the range of 62%‐100% of BD patients . In addition, there are other widespread clinical manifestations of BD, including skin lesions such as erythema nodosum and papulo‐pustular lesions, ocular involvement occurring usually within 2‐4 years of BD onset in around 20% of patients, joint involvement, gastrointestinal complications, pulmonary involvement, and cardiovascular involvement in the form of pericarditis, myocarditis, and endocarditis . Another common clinical characteristic of BD is vasculitis, which impairs small, medium, and large blood vessels (both arteries and veins) all over the body and is observed in about 15%‐38% of patients .…”

“…Current research suggests the involvement of immune cells in the pathogenesis of BD . The divergent and complicated cascade of interactions between different cytokines, particularly tumor necrosis factor (TNF)‐α, interferon‐ γ, different interleukins, chemokines, and other components of the host immune system are known to be associated with BD . The treatment of BD depends on the extent and severity of the disease .…”