musculoskeletal and connective tissue disorders
Behçet Disease
Treatment of Behçet Disease
Colchicine,dapsone,azathioprine,apremilast,thalidomide, anti-tumor necrosis factor (TNF) drugs, and/or interferon for mucosal disease Azathioprine,methotrexate,cyclosporine, or anti-TNF drugs for eye disease Anti-TNF drugs,cyclophosphamide, andchlorambucilfor refractory, severe central nervous system, or life-threatening disease Treatment of Behçet disease depends on the clinical manifestations. Treatment recommendations are limited by incomplete data from clinical studies (eg, cross-sectional studies, usually not prospective, limited statistical power). 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 central nervous system manifestations respond to high-dose systemic corticosteroids (eg,prednisone60 to 80 mg orally once a day). Anti-TNF drugs appear to be effective for a wide range of manifestations, including gastrointestinal manifestations and ocular disease (eg, severe refractory uveitis), and decrease the number of attacks. In severe cases of gastrointestinal and ocular attacks, an anti-TNF drug can be used in combination with another drug such asazathioprine.Infliximab, in particular, has the advantage of rapid onset of action. Immunosuppressants, including anti-TNF drugs, improve the prognosis for patients with vascular involvement. Immunosuppressants help prevent recurrence of venous thrombosis, but it is unclear whether anticoagulation does. Anticoagulation is contraindicated in patients with pulmonary arterial aneurysms. Mucosal disease can be managed symptomatically. Topical corticosteroids, local anesthetics, andsucralfateare helpful. Colchicine0.6 mg orally twice a day may decrease the frequency and severity of oral or genital ulcers and may be effective for erythema nodosum and arthralgias.Colchicine, which was hypothesized to decrease the need for later immunosuppressive therapy when used early in the disease course, has not been shown to do so. 50 to 100 mg orally once a day may decrease the number, duration, and frequency of oral and genital lesions. Patients should first be tested for Diagnosis . Azathioprine2.5 mg/kg orally once a day has been shown to improve oral and genital ulcers. Apremilast, an oral phosphodiesterase type 4 inhibitor, has been shown to decrease the number of oral ulcers and pain. The anti-TNF drugetanercept, 50 mg subcutaneously once a week or 25 mg subcutaneously twice a week, may suppress mucocutaneous lesions.Etanerceptcan be given ifcolchicineis ineffective. Sometimes another anti-TNF drug (infliximaboradalimumab) is used instead ofetanercept. Thalidomide100 to 300 mg orally once a day is rarely used to treat oral, genital, and skin lesions, but lesions may recur when treatment is stopped. Interferon alfa-2a 6 million units 3 times a week can also be given ifcolchicineis ineffective. Azathioprine2.5 mg/kg orally once a day helps preserve visual acuity and prevent new eye lesions.Azathioprineis also useful for mucocutaneous lesions and arthralgia. Methotrexate15 to 25 mg orally once a week has been useful in reducing eye inflammation. Cyclosporine5 to 10 mg/kg orally once a day may be reserved for patients with severe ocular manifestations and may be used withazathioprineto treat refractory uveitis. Interferon alfa-2a 6 million units subcutaneously 3 times a week orinfliximab(a TNF inhibitor) 3 to 10 mg/kg IV at 0, 2, and 4 weeks and then every 8 weeks appear promising for patients with ocular manifestations. Cyclophosphamide,chlorambucil, and anti-TNF drugs are used in patients with refractory disease, life-threatening conditions (eg, pulmonary aneurysms), or central nervous system manifestations. A trend toward longer event-free survival has been observed in patients with severe neurologic manifestations after treatment with IVcyclophosphamidethan withazathioprine. 