behçets disease Flashcards

1
Q

what

A

complex inflammatory condition

characteristically presenting with recurrent oral and genital ulers

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2
Q

link with what gene

A

HLA B51

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3
Q

prognostic indicator of severe disease

A

HLA B51

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4
Q

features: mouth ulcera

A

3 episodes per year

painful, circumscribes w red halo

heal over 2-4wks

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5
Q

features: genital ulcers

A

kissing ulcers

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6
Q

features: skin

A

erythema nodosum
papules + pustules
vasculitic type rashes

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7
Q

features: eyes

A

anterior or posterior uveitis
retinal vasculitis
retinal haemorrhage

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8
Q

features: MSK

A

AM stiffness
arthralgia
oligoarthritis usually knee or ankle

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9
Q

features: GI

A

inflammation affecting

  • ileum
  • caecum
  • asc colon
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10
Q

features: CNA

A

memory impairment
headache
aseptic meningitis
meningoencephalitis

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11
Q

features: veins

A

budd chiari syndrome
DVT
thrombus in pulmonary veins
cerebral venous sinus thrombosis

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12
Q

features: lungs

A

pulmonary artery aneurysms can develop

if rupture can be fatal

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13
Q

diagnosis based on

A

clinicla features

Ix = pathergy test

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14
Q

pathergy test

A

sterile needle to create s/c abrasion on forearm

review it 24-48hrs after to look for a weal 5mm or more

tests for non-specific hypersensitivity of skin

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15
Q

when can pathergy test be positive

A

behcet’s disease
sweets syndrome
pyoderma gangrenosum

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16
Q

Mx

A
topical and systemic steroids
colchicine
topical anaesthetics
immunosuppresion 
biologics
17
Q

prognosis

A

relapsing remitting condition

normal life expectancy and can go into remission

inc mortality with haemoptysis, neurolgical involvement