Behcet Disease

Posted by e-Medical PPT
Behçet's disease is a rare immune-mediated systemic vasculitis that often presents with mucous membrane ulceration and ocular involvements. As a systemic disease, it can also involve visceral organs such as the gastrointestinal tract, pulmonary, musculoskeletal, and neurological systems. This syndrome can be fatal, due to ruptured vascular aneurysms, or severe neurological complications.

Signs and Symptoms
Skin and mucosa
Nearly all patients present with some form of painful oral mucocutaneous ulcerations in the 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 around the anus, vulva or scrotum and cause scarring in 75% of the patients.

Ocular system
Eyes
Inflammatory eye disease can develop early in the disease course and lead to permanent vision loss in 20% of cases. Ocular involvement can be in the form of posterior uveitis, anterior uveitis, or retinal vasculitis. Anterior uveitis presents with painful eyes, conjuctival redness, hypopyon, and decreased visual acuity, while posterior uveitis presents with painless decreased visual acuity and visual field floaters.

Optic nerve involvement in Behçet's disease is rare, typically presenting as progressive optic atrophy and visual loss.

Signs and symptoms of acute optic neuropathy include painless loss of vision which may affect either one or both eyes, reduced visual acuity, reduced color vision, relative afferent pupillary defect, central scotoma, swollen optic disc, macular edema, or retrobulbar pain.
Gastrointestinal tract (bowels)
GI manifestations include abdominal pain, nausea, and diarrhea with or without blood, and they often involve the ileocecal valve.
Lung involvement is typically in the form of hemoptysis, pleuritis, cough, or 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, and is usually a non-erosive poly or oligoarthritis primarily of the large joints of the lower extremities.
Neurological involvements range from aseptic meningitis to vascular thrombosis such as dural sinus thrombosis and organic brain syndrome manifesting with confusion, seizures, and memory loss.They often appear late in the progression of the disease but are associated with a poor prognosis.

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