Behcet's Disease PDF

BEHCET'S DISEASE BY: Amira Ali  BEHCET'S DISEASE INTRODUCTION Behçet disease is a rare vasculitic disorder that is characterized by a triple-symptom complex of recurrent oral aphthous ulcers, genital ulcers, and uveitis. EPIDEMIOLOGY  It is a rare disease (Eur...

BEHCET'S DISEASE BY: Amira Ali  BEHCET'S DISEASE INTRODUCTION Behçet disease is a rare vasculitic disorder that is characterized by a triple-symptom complex of recurrent oral aphthous ulcers, genital ulcers, and uveitis. EPIDEMIOLOGY  It is a rare disease (European countries: 0.3 to 6.4 / 100,000).  More common along Ancient Silk Road (Turkey :400/100,000).  Familial clustering is rare in Europe, but has been described in up to 18 % of Turkish  3rd decade of life.  Male : female 5 : 1 ETIOLOGY:  Unknown but may be due to  Genetic cause: HLA antigen B51.  Environmental causes: infections, smoking…etc. CLINICAL MANIFESTATIONS Mucocutaneous involvement Oral aphthae: Painful and vary in size. Genital aphthae: last longer than oral lesions, are deeper, and scar after healing. Erythema nodosum: common in females. Others: Papulo-pustular lesions and superficial thrombophlebitis. Pathergy Test: highly specific for Behcet's disease. Ocular inflammation:  Typically anterior uveitis associated with:  Hypopyon  Panuveitis with posterior chamber involvement  Retinal vasculitis and associated complications. 1  Central nervous system involvement:  Aseptic meningitis or parenchymal lesions can result in focal or diffuse brain dysfunction.  Focal or multifocal nervous system involvement reflects the predilection of BS for diencephalon, midbrain, and brainstem.  Closely mimics multiple sclerosis. Muscloskeletal manifestations:  Arthritis is usually intermittent, symmetric  oligoarthritis of the knees, ankles, hands, or wrists  Arthralgia is also common. Cardiovascular manifestations:  Cerebral venous thrombosis  Deep venous thrombosis  Arterial complications as stenoses, occlusions, and aneurysms. Gastrointestinal (GI):  Symptoms include melena and abdominal pain.  GI tract inflammation can mimic inflammatory bowel disease (IBD). Oral Ulcer Genital ulcers Erythema nodosum Papulopustular lesions Pathergy test 2 CLASSIFICATION CRITERIA FOR BEHCET’S DISEASE Criteria Definition Recurrent oral ulcers At least 3 episodes at 12 months period Plus 2 of the following Eye lesion Anterior/posterioruveitis/retinal vasculitis Skin lesion Erythema nodosum/pseudofolliculitis Pathergy test Interpreted at 24-48 hours LABORATORY FEATURES Acute-phase reactants: May be normal or increased. HLA B51: Associated with BS in areas of high prevalence and in patients with ocular disease. 3 TREATMENT Glucocorticoids: Aphthous lesions: topical or intralesional. Colchicine:  For muscloskeletal msnifestations.  GI intolerance can occur at higher doses. Thalidomide: For mucocutaneous lesions, but toxicity is a major concern. Glucocorticoid With immunosuppressive agents for severe disease. combination therapy: Pulmonary arterial Cerebral venous The treatment of aneurysms thrombosis Budd–Chiari syndrome may respond to the is treated with includes combination of anticoagulation and anticoagulants and prednisone and glucocorticoids. immunosuppressive cyclophosphamide. medications. Cyclosporine and  Monotherapy or in combination to treat uveitis. azathioprine:  Azathioprine can have a beneficial effect on mucosal ulcers, arthritis, DVT, and long-term prognosis. Tumor necrosis Particularly posterior uveitis and CNS disease. factor (TNF) inhibitors: DMARDS BIOLOGICS colchicine steroid -Azathioprine Cyclophosphamide -TNF Blockers -Mycophenolate -Rituximab mofetil 4