Bechets Disease

Question 1

What is Behcets disease?

Answer 1

Systemic vasculitis characterized by deposition of immune complexes in arteries + veins of all sizes
Often presents with orogenital ulceration + uveitis

Question 2

Describe the aetiology of Behcets disease

Answer 2

UNKNOWN cause
-ve ANCA
H.pylori/ HSV/ Parvovirus may be involved

Question 3

Which MHC is Behcets disease associated with?

Answer 3

HLA-B51 (AI)

Question 4

Describe the epidemiology of Behcets disease

Answer 4

More common in Turkey, Greece + Central Asia

Peak in 20-30s

Question 5

Describe the main presentation of Behcets disease

Answer 5

Recurrent ORAL + GENITAL ulceration

Question 6

What genital manifestations usually arise in Behcets disease? Which sites are most commonly involve?

Answer 6

Single or multiple ulcers that resemble oral pathos ulcers + heal with scarring
M: Scrotum
F: Vulva

Question 7

What oral manifestations usually arises in Behcets disease?

Answer 7

Painful Apthous Ulcers
In moist mucosal surfaces inside the mouth
Do NOT occur on outer surfaces of the lips

Question 8

What CNS symptoms may arise in Behcets disease?

Answer 8

memory loss, headache, confusion, impaired speech/ balance/ movement

Question 9

What skin manifestations may arise in Behcets disease?

Answer 9

Erythema nodosum
Pyoderma gangrenosum
Acne lesions

Question 10

What ocular manifestations may arise in Behcets disease?

Answer 10

Uveitis => eye pain, blurry vision, photophobia, redness, photosensitivity
Hypopyon: leukocytes in anterior chamber of the eye

Question 11

What joint manifestations may arise in Behcets disease?

Answer 11

Asymmetric, non erosive Arthritis

Question 12

What vascular manifestations may arise in Behcets disease?

Answer 12

Vasculitis

Thrombophlebitis

Question 13

What GI manifestations may arise in Behcets disease?

Answer 13

Colitis:
Abdo pain
Anorexia
Diarrhoea 
N + V

Question 14

What CVS manifestations may arise in Behcets disease?

Answer 14

Myocarditis

Pericarditis

Question 15

What specific investigation is performed in Behcets disease?

Answer 15

Pathergy Test: A sterile erythematous papule or pustule develops 48 hours after a needle prick to a depth of 5 mm

Question 16

What investigations may be performed if there is suspicion of CNS, GI or Resp involvement exclude other causes?

Answer 16

CNS: CT/MRI head
GI: colonoscopy/ endoscopy
Resp: CT chest (or angiography) to screen for pulmonary aneurysm

Question 17

Describe the features of Behcets disease on colonoscopy

Answer 17

Distinct, single, deep ulcers.

inflammation + aphthous-type ulcers, possibly vasculitis on biopsy

Question 18

What general investigations may be performed in Behcets disease?

Answer 18

Measure complement levels
Check for a positive FH
Exclude other AI conditions e.g. test for ANA, anti-CCP