- Behçet’s disease is a multi-system inflammatory disorder of unknown aetiology.
- It typically affects patients aged 20–40, with ethnicities linked to the ancient silk-road trading route—from modern day Turkey to Iran, China, Korea and Japan.
- Behcet’s disease is characterized by widespread mucosal ulceration and vasculitis, thought to be due to abnormal lymphocyte and cytokine production leading to vascular endothelial dysfunction
- Clinical
- Typical
- mouth and genital ulceration, uveitis, retinal vasculitis and arthritis
- Gastrointestinal manifestation
- Bowel ulceration, typically in the ileocaecal region, most commonly presenting as pain, nausea, diarrhoea and bleeding.
- Patients may present with mesenteric vasculitis causing aneurysms requiring repair or thrombosis resulting in bowel ischaemia requiring resection.
- Diagnosis
- On biopsy of GI ulcer they lack the histopathological features of Crohn’s disease, although may result in similar complications including strictures, perforation and fistula formation.
- Although no diagnostic test exists for Behçet’s disease, and a clinical diagnosis is usually made in the presence of sufficient symptoms, HLA B51 and HLA A26 positivity can be helpful.
- Management of acute ulcerative gastrointestinal disease is with glucocorticoids and other therapies such as immunomodulators or TNF-alpha inhibitors.
- Gastrointestinal surgery should be avoided where practical due to high recurrence rates of ulcers post-operatively. Instead, medical management in consultation with a gastroenterologist should be considered first line of treatment.