Behçet disease

Posted by e-Medical PPT
Behçet disease is a rare, systemic, form of vasculitis that often presents with mucous membrane ulceration, and ocular involvements.As a systemic disease, it also involves visceral organs such as the gastrointestinal tract, pulmonary, musculoskeletal, and neurological systems. This syndrome can be fatal; death can be caused by complicated rupture of the vascular aneurysms, or severe neurological complications, and therefore immediate medical treatment is necessary.
The etiology is not well-defined, but it is primarily characterized by auto-inflammation of the blood vessels.
Nearly all patients present with some form of painful oral mucocutaneous ulcerations in form of aphthous ulcers or non-scarring oral lesions.The oral lesions are similar to those found in inflammatory bowel disease and can be relapsing.Painful genital ulcerations usually develop on the vulva and the scrotum and cause scarring in 75% of the patients.Inflammatory eye disease can develop early in the disease course and lead to permanent vision loss in 20% of the cases.Ocular involvement can be in form of posterior uveitis, anterior uveitis, or retinal vasculitis.Lung involvement is typically in form of hemoptysis, pleuritis, cough, fever, and in severe cases can be life threatening if the outlet pulmonary artery develops an aneurysm which ruptures causing severe vascular collapse and death from bleeding in the lungs.Arthralgia is seen in up to half of patients.
Current treatment is aimed at easing the symptoms, reducing inflammation, and controlling the immune system. High dose Corticosteroid therapy (1 mg/kg/d oral prednisone) is indicated for severe disease manifestationsAnti-TNF therapy such as infliximab has shown promise in treating the uveitis associated with the disease

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