One year in review 2016: Behçet's syndrome

Area of Science:

• Rheumatology
• Immunology
• Genetics

Background:

• Behçet's syndrome (BS) is a multisystem inflammatory disorder with complex pathogenesis.
• Recent literature reviews highlight advancements in understanding BS epidemiology, clinical manifestations, and treatment.

Purpose of the Study:

• To synthesize recent findings on Behçet's syndrome (BS) across various domains.
• To provide an updated overview of diagnostic criteria, clinical features, and therapeutic strategies for BS.

Main Methods:

• Review of recently published articles on Behçet's syndrome (BS).
• Analysis of clinical, radiological, and genetic studies.
• Evaluation of treatment outcomes from uncontrolled and phase III trials.

Main Results:

• Distinctive features of BS-related deep vein thrombosis and increased post-thrombotic syndrome risk.
• Clinical and colonoscopic markers aid differentiation from Crohn's disease; Barkhof criteria assist in distinguishing neurological involvement from multiple sclerosis.
• Anatomical localization of papulopustular lesions is more useful than histology for differentiating from acne vulgaris.
• Contradictory results on ovarian reserve; increased hematological malignancy risk noted in Taiwanese female BS patients.
• Genetic factors (ERAP, HLA B51 interactions) and microbiome data are emerging.
• Anti-TNF therapies show promise for refractory extra-ocular complications; IL-1 inhibition shows potential, though a specific anti-IL-1β antibody failed a phase III trial.
• Ongoing debate regarding anticoagulation in BS.

Conclusions:

• Recent research deepens the understanding of Behçet's syndrome (BS) pathogenesis, highlighting autoimmune and autoinflammatory aspects.
• New data inform differential diagnosis and management of BS complications, including thrombosis and extra-ocular involvement.
• Therapeutic strategies, particularly anti-TNF agents, show promise for refractory cases, while further research is needed for IL-1 inhibition and anticoagulation.