One year in review 2017: Behçet's syndrome

Area of Science:

• Ophthalmology
• Rheumatology
• Immunology
• Genetics

Background:

• Behçet's syndrome (BS) prevalence varies globally due to methodological differences in studies and true geographic factors.
• Understanding BS pathogenesis involves exploring immune system links to thrombosis and genetic associations.
• Developing standardized outcome measures for clinical trials remains an ongoing effort.

Purpose of the Study:

• To review recent advancements in understanding Behçet's syndrome.
• To highlight key diagnostic, therapeutic, and etiological findings.
• To discuss the role of imaging, genetics, and novel treatments.

Main Methods:

• Meta-analysis of prevalence studies.
• Review of multimodal imaging techniques (color fundus photography, fluorescein angiography, optical coherence tomography).
• Analysis of recent genetic studies (immunochip) and micro-RNA research.
• Evaluation of clinical trial data for biologics (anti-TNF agents, adalimumab) and other treatments (interferon-alpha, ustekinumab, anakinra, canakinumab).

Main Results:

• Methodological variations contribute to reported BS prevalence differences.
• Multimodal imaging is crucial for diagnosing and monitoring Behçet's uveitis.
• Thrombosis in BS may involve structurally altered, plasmin-resistant fibrin clots.
• HLA-B51 remains the primary genetic association, with new micro-RNA insights.
• Anti-TNF agents, particularly adalimumab, are effective for Behçet's uveitis.
• Interferon-alpha shows potential for long-lasting remission; other biologics show promise pending further study.

Conclusions:

• Advances in understanding Behçet's syndrome encompass prevalence factors, diagnostic imaging, thrombosis mechanisms, and genetic links.
• Anti-TNF therapy, especially adalimumab, is a cornerstone for managing Behçet's uveitis.
• Emerging treatments and ongoing research into immune regulation offer future therapeutic avenues.