Table 2.

Approach to the management of different organ presentations in BD

Disease manifestation	First-line treatments	Second-line treatments	Experimental treatments
Mucocutaneous	Colchicine Glucocorticoids (low-dose oral/topic)	Azathioprine Apremilast TNFα inhibitors Interferon-α	Anti-IL1 Ustekinumab Secukinumab
Articular	Colchicine Salazopyrin Methotrexate	TNFα inhibitor Interferon-α	Anti-IL-1 Secukinumab
Ocular	Azathioprine Cyclosporine A	TNFα inhibitors Interferon-α	Tocilizumab
Vascular	Azathioprine(V,A) Cyclosporine A(V) Cyclophosphamide (A)	TNFα inhibitors Interferon-α	Tocilizumab
Neurological	Azathioprine Mycophenolate mofetil	TNFα inhibitors Interferon-α Cyclophosphamide	Tocilizumab
Gastrointestinal	Salazopyrin Azathioprine	TNFα inhibitors	Anti-IL-1 Tocilizumab

V venous, A arterial

Dosing, colchicine 0.5–2 mg/day; azathioprine, 2–2.5 mg/kg/day, glucocorticoids: low-dose, 5–10 mg/day prednisolone; Salazopyrin, 0.5–3 g/day, methotrexate, 5–25 mg/week; cyclophosphamide, 0.5–1 g/month; cyclosporine A, 3–5 mg/kg/day; mycophenolate mofetil, 0.5–3 g/day; apremilast, 60 mg/day; Interferon-α, 3–9 million IU/3–7 d weekly; TNFα inhibitors: infliximab, 3–10 mg/kg/4–8 weekly, adalimumab, 40 mg/SC/1-2 weekly; IL-1 antagonists: anakinra, 100–200 mg/SC daily; canakinumab, 150–300 mg/2–4 weekly; tocilizumab, 4–8 mg/kg/4 weekly, 162 mg/weekly SC; ustekinumab, 45–90 mg/4–8 weekly; secukinumab, 150–300 mg/4–8 weekly