Treatment of Behçet's Disease

Organ-Specific Treatment Algorithm

The American College of Rheumatology recommends that treatment of Behçet's disease must be tailored to the specific organ system involved, with aggressive immunosuppression required for sight-threatening, neurological, vascular, and gastrointestinal manifestations to prevent irreversible damage and death 1

Colchicine 1-2 mg/day is the first-line treatment for arthritis and mucocutaneous lesions, particularly effective in women, according to the European League Against Rheumatism 2
The European League Against Rheumatism suggests that colchicine has proven efficacy in two randomized controlled trials for arthritis and erythema nodosum 2

The American Academy of Ophthalmology recommends that any patient with posterior segment eye disease must receive azathioprine combined with systemic corticosteroids as mandatory first-line therapy 2
For severe eye disease, the American Academy of Ophthalmology suggests adding either infliximab or cyclosporine A to the azathioprine-corticosteroid combination; alternatively, interferon-alpha with or without corticosteroids can be used 2

The American Academy of Neurology recommends that for acute parenchymal Neurobehçet's disease, initiate high-dose intravenous methylprednisolone followed by oral prednisolone, combined with azathioprine 2.5 mg/kg/day as the preferred steroid-sparing agent from the outset 1
The American Academy of Neurology suggests that TNF-alpha inhibitors, particularly infliximab, are recommended for refractory or severe cases failing conventional therapy 1
The American Academy of Neurology warns that cyclosporine A should never be used in patients with CNS involvement due to significant neurotoxicity risk 1, 2

The American Heart Association recommends that for acute deep vein thrombosis, use immunosuppressive agents (corticosteroids combined with azathioprine, cyclophosphamide, or cyclosporine A) rather than anticoagulation 2
The American Heart Association suggests that for pulmonary and peripheral arterial aneurysms, cyclophosphamide combined with corticosteroids is recommended 2
The American Heart Association warns that anticoagulants, antiplatelet agents, or antifibrinolytic agents should not be used for venous thrombosis in Behçet's disease 2

The American Gastroenterological Association recommends that before considering surgery (except emergencies), trial sulfasalazine, corticosteroids, azathioprine, TNF-alpha antagonists, or thalidomide sequentially for gastrointestinal ulcers 2

The American College of Rheumatology recommends that young men with early disease onset require more aggressive immunosuppression and closer monitoring due to higher risk of severe disease and major organ involvement 1, 4

The American College of Rheumatology recommends that for patients failing conventional immunosuppression across any organ system, TNF-alpha inhibitors (infliximab or adalimumab) represent the next therapeutic step 1
The American College of Rheumatology suggests that infliximab combined with azathioprine shows particular promise for severe or refractory cardiac involvement 3

The American College of Rheumatology recommends that disease manifestations typically ameliorate over time, allowing for treatment tapering and potential discontinuation in stable patients 1, 4
The American College of Rheumatology suggests that regular monitoring of inflammatory markers and organ-specific assessments remains essential even during remission 1, 4