Synopsis
Recognise Behcet disease from recurrent mucosal ulceration with ocular, skin, vascular, neurological and gastrointestinal inflammation, exclude infection and mimics, and treat organ-threatening disease through specialist multidisciplinary pathways.
- Behcet disease is a clinical diagnosis: recurrent painful oral ulcers plus genital ulcers or scars, eye inflammation, characteristic skin lesions, vascular, neurological or gastrointestinal disease forms the pattern.
- No single blood or genetic test confirms Behcet disease; HLA-B51 supports population susceptibility but has insufficient diagnostic specificity.
- Document oral and genital lesions with consent, frequency, scarring and negative infection tests; ask directly because lesions may have healed before review.
- Use the 2014 International Criteria to organise evidence, but exclude HSV, syphilis, HIV, inflammatory bowel disease, reactive disease, lupus and malignancy.
- Any visual symptom needs same-day ophthalmology; topical drops alone are inadequate for posterior uveitis or retinal vasculitis.
- Colchicine is first-line for recurrent genital ulcers and erythema nodosum and can help arthritis; topical corticosteroid treats individual mucosal ulcers.
- Azathioprine, ciclosporin, interferon-alpha or anti-TNF therapy is selected for ocular or systemic disease; avoid ciclosporin in neurological involvement.
- Inflammatory venous thrombosis is treated primarily with immunosuppression; anticoagulation is individualised only after excluding pulmonary-artery aneurysm and assessing bleeding risk.
- Pulmonary-artery aneurysm with haemoptysis requires high-dose glucocorticoid plus cyclophosphamide or anti-TNF and interventional planning, not routine anticoagulation alone.
- Coordinate contraception and pregnancy: colchicine and azathioprine may be compatible, while methotrexate, mycophenolate and cyclophosphamide require avoidance or specialist cessation.
Key red flags
A painful photophobic eye, floaters or reduced vision requires same-day slit-lamp and retinal assessment because posterior uveitis can rapidly blind.
Haemoptysis, chest pain or pulmonary-artery aneurysm requires emergency respiratory and vascular imaging before anticoagulation.
Severe headache, papilloedema, focal deficit, confusion or seizure suggests cerebral venous thrombosis or neuro-Behcet disease.
Painful swollen limb, vena-caval obstruction or Budd-Chiari syndrome reflects inflammatory thrombosis and needs combined immune and vascular care.
Deep ileocaecal ulceration can bleed or perforate and requires urgent gastroenterology and surgery.
Photophobia, floaters, blurred vision or field change may indicate anterior or posterior uveitis and occlusive retinal vasculitis.
Haemoptysis or focal arterial pain can indicate pulmonary or systemic aneurysm and carries rupture risk.
Investigation priorities
Establish recurrence and combine mucosal, ocular, skin, vascular and neurological evidence.
Management branches
Recurrent oral ulcers coexist with genital, ocular, skin, vascular, neural or gut features.
- Document episode frequency, photographs, scars and objective specialist findings and screen sexual, medicine, bowel and inflammatory mimics.
- Obtain baseline blood and organ tests and urgently image or refer any eye, neurological, vascular or abdominal red flag.
- Apply criteria as support, then agree an organ-by-organ diagnosis, damage record and rapid-access plan with a Behcet-experienced team.
Major venous thrombosis, pulmonary aneurysm or arterial occlusion occurs.
- Image arterial and venous territories, explicitly excluding pulmonary aneurysm before any anticoagulant decision and assess haemorrhage and organ ischaemia.
- Use high-dose glucocorticoid with cyclophosphamide or anti-TNF for severe arterial or major venous disease and involve interventional teams.
- Individualise anticoagulation for selected venous thrombosis only after aneurysm and bleeding review and continue imaging until vessel inflammation and anatomy stabilise.