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 Behcet's Syndrome Treatment
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James Minor
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Behcet's Syndrome Treatment 

  • Colchicine , thalidomide, etanercept  and interferon  for mucosal  disease
  • Azathioprine or cyclosporine for eye  disease
  • Cyclophosphamide  and chlorambucil  for refractory or life-threatening disease 

Treatment depends on the clinical manifestations. 

Mucosal disease can be managed symptomatically. 

Colchicine 0.6 mg po bid may decrease  the frequency  and severity  of oral  or genital ulcers  and may be effective for erythema nodosum  and arthralgias. 

Thalidomide 100  to 300 mg po once/day may be  used to treat oral, genital, and  skin lesions, but lesions may recur when treatment is stopped. 

Etanercept 50 mg sc once/wk or 25 mg sc twice/wk may  suppress mucocutaneous lesions. Etanercept  can be given in colchicine is ineffective. Interferon-α2a 6 million units 3 times/wk  can also be given if  colchicine is ineffective. 

Azathioprine 2.5 mg/kg once/day helps preserve visual acuity  and prevent new eye lesions. Azathioprine  is also useful for mucocutaneous lesions  and arthralgia. 

Cyclosporine 5  to 10 mg/kg once/day  may be reserved  for patients with severe ocular manifestations  and may be  used with azathioprine to treat refractory uveitis. 

Interferon-α2a 6 million units 3 times/wk  and infliximab (a tumor necrosis factor inhibitor) 3  to 10 mg/kg at 0, 2, 4, and then every 8 wk show promise for  patients with ocular manifestations. 

Cyclophosphamide and chlorambucil  are used  in patients with refractory disease, life-threatening  conditions (eg, pulmonary aneurysms),  or CNS manifestations. 

The efficacy  of corticosteroids  is unsubstantiated, despite their wide use. Topical corticosteroids  may temporarily relieve ocular manifestations and most oral lesions. 

However,  topical or systemic  corticosteroids do not alter  the frequency of relapses. A few patients with severe uveitis  or CNS manifestations respond  to high-dose systemic corticosteroids (eg,  prednisone 60  to 80 mg po once/day). 

Whether immunosuppressants should be added  to anticoagulation therapy when patients have thromboses  has not been established. 

Notes:
DrJMinor
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EditText of this page (last edited December 19, 2009)

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