Behcet's syndrome is a complex multisystem disorder associated with presumed autoimmune-mediated inflammation of the arteries and veins. The precise aetiology has yet to be elucidated however.
Epidemiology
- more common in the eastern Mediterranean (e.g. Turkey)
- more common in men (complicated gender distribution which varies according to country. Overall, Behcet's is considered to be more common and more severe in men)
- tends to affect young adults (e.g. 20 - 40 years old)
- associated with HLA B51
- around 30% of patients have a positive family history
PC
- classically: 1) oral ulcers 2) genital ulcers 3) anterior uveitis
- thrombophlebitis and deep vein thrombosis
- arthritis
- neurological involvement (e.g. aseptic meningitis)
- GI: abdo pain, diarrhoea, colitis
- erythema nodosum
- can cause acute pericarditis
Diagnosis