Several studies were published last year which focused on the epidemiology, 
immunopathogenesis, genetics, clinical manifestations and management of Behçet's 
syndrome. Recent epidemiologic studies support the earlier contention that the 
frequency of BS increases from North to South in Europe, BS is rare in 
Sub-Saharan Africa, it follows a more severe course among young men, especially 
if the disease onset is at a young age and that in European countries, the 
frequency is higher among immigrants from BS prevalent countries compared to 
locals living in the same environment. The relationship between HLA-B51 and 
Behçet's was re-emphasised and a functional role affecting cellular cytotoxicity 
was proposed. Innate immunity was explored and TLR7 copy number variations and 
nucleic acid sensors of varying inflammasome pathways were studied. Vascular 
relapse risk is decreased when BS patients are treated with immunosuppressives 
with or without anti-coagulation rather than anti-coagulation alone. Although 
rare in the Far East, the clinical picture of the vascular involvement was quite 
similar to the previously published reports. Interestingly a female predominance 
among those with cerebral vein thrombosis was noted. Venous claudication is a 
frequent and severe symptom among BS patients with lower extremity DVT. 
Budd-Chiari syndrome associated with BS is usually associated with IVC 
thrombosis. Silent cases exist and have a better prognosis. The mortality rate 
among the patients symptomatic for liver disease remains high. Methotrexate 
seems to be effective in the treatment of chronic progressive neuro-Behçet's 
disease. Renal involvement is an uncommon disorder in BS. Suicidal thoughts are 
increased among BS patients with severe organ involvement. Work-related 
disability in BS is high and under-appreciated. Apremilast, an inhibitor of 
phosphodiesterase-4, was effective in a phase 2, double blind, 
placebo-controlled study. Adalimumab seems to be effective in severe uveitis of 
BS even after failure of infliximab. New cytokine inhibitors targeting IL-1 and 
IL-6 appear to be effective especially for uveitis and CNS involvement 
refractory to anti TNF agents.