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.
