Behcet's Syndrome Flashcards
Behcet’s Syndrome
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. The classic triad of symptoms are oral ulcers, genital ulcers and anterior uveitis
Behcet’s Syndrome - Classic TRIAD
ORAL ULCERS
GENITAL ULCERS
ANTERIOR UVEITIS
Behcet’s Syndrome - Epidemiology
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 B5* and MICA6 allele
around 30% of patients have a positive family history
*more specifically HLA B51, a split antigen of HLA B5
Behcet’s Syndrome - Features
Features
classically: 1) oral ulcers 2) genital ulcers 3) anterior uveitis
thrombophlebitis
arthritis
neurological involvement (e.g. aseptic meningitis)
GI: abdo pain, diarrhoea, colitis
erythema nodosum, DVT
Behcet’s Syndrome - Diagnosis
Diagnosis
no definitive test
diagnosis based on clinical findings
positive pathergy test is suggestive (puncture site following needle prick becomes inflamed with small pustule forming)
Behcet’s Syndrome - Diagnosis: Example Question
A 32-year-old gentleman presented to his GP with an 8-week history of debilitating pain in his oral cavity and in his groin area. He had presented on numerous occasions to his GP with episodes of tiredness and non-specific malaise which each time was put down to non-specific viral illness. Eight months ago he was investigated by a gastroenterologist having presented with bloody diarrhoea and abdominal pain. He was diagnosed with a non-specific colitis of unknown origin which resolved spontaneously. He also suffered a solitary DVT of his left leg 6 years ago which was treated with oral anticoagulation. He smoked 20 cigarettes per day and consumed 20 units of alcohol per week. He was on no regular medication. Upon specific questioning, he denied joint pain or swelling. He also denied the presence of back pain. He was unaware of any family history as he was adopted from birth.
On examination, he appeared pale. His heart rate was 88 and blood pressure 118/78 mmHg. Examination of his cardiovascular system was unremarkable. Examination of his abdomen was likewise unremarkable. Examination of his oral mucosa revealed the presence of multiple aphthous ulceration. Examination of his external genitalia likewise revealed the presence of multiple shallow ulcers within his groin region. Examination of his joints was unremarkable.
Initial investigations revealed the following results:
Hb 139 g/l Platelets 333 * 109/l WBC 5.1 * 109/l ESR 22 mm/hr CRP 28 mg/l Rheumatoid factor negative Anti CCP negative ANA negative HLA B27 positive
What is the most likely underlying diagnosis?
Seronegative arthritis Disseminated gonococcal infection Crohn's disease Coeliac disease > Behcet's syndrome
This gentleman presents with a combination of malaise, oral and genital ulceration, colitis and iritis. Of the above options, Behcets syndrome is the only option that unifies this combination. Note that HLA B27 is positive in 10 % of the population in the absence of seronegative arthritis. Crohn’s disease may present with a colitis and aphthous ulceration as well as iritis, but it would be difficult to account for the past deep vein thrombosis.
