behcet's disease

Question 1

definition

Answer 1

A systemic vasculitis, first described by Hulusi Behcet, a Turkish dermatologist, Behcet’s syndrome is most commonly seen in Turkey, Israel, the Mediterranean basin, and eastern Asia (where Japan and South Korea report the most cases). It can cause skin and mucosal lesions, uveitis, major arterial and venous vessel disease, and GI and neurological manifestations. These can present in various combinations and sequences in patients over time.

Question 2

epidemiology

Answer 2

20-30 y/o

more active and severe in males

Question 3

pathogenesis

Answer 3

unlike other auto-immune diseases, Behcet’s syndrome is not typically associated with auto-antibodies, Raynaud’s disease, Sjogren’s syndrome, thrombocytopenia, haemolytic anaemia, sun hypersensitivity, serosal involvement, or increased risk of other auto-immune diseases. Some features that separate it from auto-inflammatory conditions include tendency to abate with time, absence of mutations associated with auto-inflammatory diseases, and higher prevalence than typical auto-inflammatory diseases such as familial Mediterranean fever. Both innate and adaptive immune systems may be involved

Question 4

symptoms

Answer 4

• oral ulcers (on the moist inner surfaces of mouth)
• genital ulcers (penis usually spared)
• uveitis (leading cause of morbidity)
• acne lesions on arms and legs
• erythema nodosum on lower limbs (usually removes in 1-2 weeks)
• superficial thrombophlebitis (Associated with DVT and pulmonary aneurysm formation.)
• hypopyon (inflammation of anterior chamber of the eyes)

symptoms usually last 1-2 weeks

Question 5

risk factors

Answer 5

20-40 y/o
family history (there is also genetic anticipation - next generation gets symptoms earlier and more severe)
genetic predisposition

Question 6

investigations

Answer 6

1st investigations:

• pathergy testing => papule within 48hr
• ANA => should be neg
• RF => should be neg
• ANCA => should be neg
• HLA-B51 => +ve
• MRI brain w contrast (when large vessel phlebitis or CNS involvement is suspected)
• colonscopy
• upper GI endoscopy
• CT chest (when haemoptysis occurs to evaluate for pulmonary aneurysm)
• CT angio chest (when haemoptysis occurs to evaluate for pulmonary aneurysm)
• pulmonary angio