Behcet syndrome

Question 1

What are the common clinical features (not systemic) of Behcet’s disease?

Answer 1

Recurent, painful mucocutaneous ulcers

1. Oral ulcers - involves entire GIT
2. Genital ulcers (more specific)
3. Ocular disease - remember vision**** important for long case
4. Rash

Question 2

What are the systemic features of Behcet’s?

Answer 2

1. Angio-Bechet’s: Vasculitis involving all size of arteries and veins: IHD, pulmonary artery, PVD, DVT/PE
2. Neuro Behcet: causes parenchymal disease in CNS
3. Arthritis - mainly large joints
4. Skin lesions: folliculitis, erythemar nodosum, pathergy

Question 3

Greatest mortality from Behcet’s results from what?

Answer 3

1. Occular disease - remember vision
2. Vascular disease
3. CNS disease - aseptic meningoencephalitis** (remember flynn), cerebral vasculitis

Question 4

Treatment of Behcet’s?

Answer 4

1. Topical steroids + anaesthetics: oral and genital ulcers and ocular disease.
   - Regular ophthal F/U
   - Regularly assess fitness for driving
   - Sight threatening disease req pulse methyl-prednisolone
2. Colchicine*** (remember Flynn) - 1-2mg/d for prevention of recurrent disease. Also used in arthritis without other systemic features.
3. Apremilast (if above 2 not working)
4. Systemic steroids in refractory disease
5. Other steroid sparing: AZA, MTX, MMF, Cyclophosphamide