Review Article

New Evidence-Based Treatment Approach in Behçet's Disease

Table 1

Activity spectrum of systemic therapeutic agents on Behçet’s disease in randomized, controlled studies.

TreatmentDoseIndication and reference

Corticosteroids versus placebo40 mg/every 3 wDecrease the frequency of EN in women [36]
Colchicine versus placebo1–2 mg/dDecreases the frequency of EN and effective on arthralgia [37]
Reduces the occurrence of GUs, EN, and arthritis in women and the occurrence of arthritis in men [38]
1 mg/dDecrease in overall disease activity index and significant improvement in OUs, GUs, PPLs, and EN [39]
Colchicine versus Colchicine + Benzathine penicillin1–2 mg/d; 1.2 MU/3 wCombined treatment more effective in reducing frequency of arthritic episodes, duration and frequency of OUs and EN, and the frequency of GUs [40]
Colchicine versus Benzathine penicillin versus Colchicine + Benzathine penicillin1 mg/d; 1.2 MU/moCombined use of colchicine and benzathine penicillin treatment more effective than colchicine or penicillin alone [41]
Rebamipide versus placebo300 mg/dReduces the number of OUs and pain [42]
Zinc sulfate versus placebo300 mg/dSignificant improvement in the clinical manifestations index of mucocutaneous lesions [43]
Dapsone versus placebo100 mg/dEffective on the number, healing time and frequency of OUs, number of GUs, and frequency of EN and PPLs. Suppresses arthritis and epididymitis [44]
Thalidomide versus placebo100–300 mg/dSustained remission of OUs, GUs, and PPLs [45]
Azathioprine versus placebo2,5 mg/kg/dReduces the occurrence of OUs, GUs, arthritis, and ocular symptoms. Prevents the development of new eye disease [46]
Cyclophosphamide + Corticosteroids versus Corticosteroids1 g/m2/moCombined treatment of CCP and corticosteroids more effective in eye disease than corticosteroids alone [47]
Cyclosporin A versus Colchicine10 mg/kg/dCyA more effective on the severity and frequency of OUs, GUs, and PPLs. Superior to colchicine in decreasing the frequency and severity of ocular attacks [48]
Cyclosporin A versus conventional treatments (prednisolon, chlorambucil)10 mg/kg/dCsA more effective than conventional therapy in ocular disease, however, conventional therapy superior to CyA in controlling OUs, GUs, and arthritis [49]
Cyclosporin A versus conventional treatments (prednisolon, chlorambucil)10 mg/kg/dImprovement of hearing loss in 25% of patients receiving CyA treatment [50]
Cyclosporin A versus Cyclophosphamide5 mg/kg/dA significant improvement in VA during the first 6 months in CyA group compared with CCP [51]
Cyclosporin A versus conventional treatments (prednisolon, Azathioprine)5 mg/kg/dCyA more effective than conventional therapy in OUs, GUs, cutaneous lesions, thrombophlebitis as well as articular and neurologic symptoms [52]
Interferon-α versus placebo6 MU/d-3 x/wEffective on pain and healing time of OUs and frequency of GUs and PPLs. Also helpful in decreasing frequency and duration of EN, TFB, and articular symptoms [54]
Etanercept versus placebo25 mg/d-2 x/wReduces the occurrence of OUs, nodular skin lesions, and PPLs [64]
Rituximab versus cytotoxic combination therapy2 1000-mg courses (15-day interval)A significant improvement in total adjusted disease activity index in rituximab group [65]

d: day; EN: erythema nodosum; GUs: genital ulcers; Mo: month; OUs: oral ulcers; PPLs: papulopustular lesions; TFB: thrombophlebitis; VA: visual acuity; w: week.