Table 4 EULAR general treatment recommendations for the treatment of Behçet’s disease

Any patient with BD and inflammatory eye disease affecting the posterior segment should be on a treatment regime that includes azathioprine and systemic corticosteroids

If the patient has severe eye disease defined as >2 lines of drop in visual acuity on a 10/10 scale and/or retinal disease (retinal vasculitis or macular involvement), it is recommended that either cyclosporine A or infliximab be used in combination with azathioprine and corticosteroids. Alternatively IFN-α with or without corticosteroids could be used instead

There is no firm evidence to guide the management of major vessel disease in BD. For the management of acute deep vein thrombosis in BD, immunosuppressive agents such as corticosteroids, azathioprine, cyclophosphamide or cyclosporine A are recommended. For the management of pulmonary and peripheral arterial aneurysms, cyclophosphamide and corticosteroids are recommended

Similarly, there are no controlled data on, or evidence of benefit from uncontrolled experience with anticoagulants, antiplatelet or anti-fibrinolytic agents in the management of deep vein thrombosis or for the use of anticoagulation for the arterial lesions of BD

There is no evidence-based treatment that can be recommended for the management of gastrointestinal involvement of BD. Agents such as sulfasalazine, corticosteroids, azathioprine, TNF-α antagonists and thalidomide should be tried first before surgery, except in emergencies

In most patients with BD, arthritis can be managed with colchicine

There are no controlled data to guide the management of CNS involvement in BD. For parenchymal involvement, agents to be tried may include corticosteroids, IFN-α, azathioprine, cyclophosphamide, methotrexate and TNFα antagonists. For dural sinus thrombosis, corticosteroids are recommended

Cyclosporine A should not be used in BD patients with central nervous system involvement unless necessary for intraocular inflammation

The decision to treat skin and mucosa involvement will depend on the perceived severity by the doctor and the patient. Mucocutaneous involvement should be treated according to the dominant or codominant lesions present. Topical measures (i.e., local corticosteroids) should be the first line of treatment for isolated oral and genital ulcers. Acne-like lesions are usually of cosmetic concern only. Thus, topical measures as used in acne vulgaris are sufficient. Colchicine should be preferred when the dominant lesion is erythema nodosum. Leg ulcers in BD might have different causes. Treatment should be planned accordingly. Azathioprine, IFN-α and TNF-α antagonists may be considered in resistant cases