Therapeutic approach to central nervous system involvement of Behçet's disease

Background: Neurologic involvement in Behçet's disease (BD) represents a major cause of disease morbidity and mortality. Early recognition and timely treatment represent crucial aspects that aim at preventing long-term disability. The absence of robust and evidence-based studies further complicates the management of neuro-BD (NBD). In this review we aim at collecting the best available evidence and suggest a treatment algorithm for an optimal and personalized management of NBD. Evidence acquisition: PubMed (NLM) database for papers written in English language was used to retrieve relevant articles for this review. Results and conclusions: Neurologic involvement in BD is one of the most serious and challenging aspects to manage, particularly in its chronic progressive form. It is important to distinguish between acute and chronic progressive NBD, as treatment may vary considerably. Currently, no standardized treatment guidelines support physicians in the decision-making process that therefore relies on low-level evidence. High dose corticosteroids remain the cornerstone for managing acute phase both in the parenchymal and non-parenchymal involvement. Prevention of relapses and control of disease progression represent crucial goals for acute and chronic progressive NBD respectively. In this regard, mycophenolate mofetil and azathioprine are valuable options in the acute NBD. On the other hand, low weekly dose methotrexate has been suggested for chronic progressive NBD. Refractory cases or intolerant patients to conventional therapies may benefit from biologic agents, particularly infliximab. First-line infliximab may be preferred in severe patients with high risk of damage. Other agents including tocilizumab, interleukin-1 inhibitors, B-cell depletion therapy and to a lesser extent, interferon-α and intravenous immunoglobulins are potential options in severe and multidrug resistant cases. Due to multiple organ involvement in BD, long-term treatment should be determined by a multidisciplinary approach. Therefore, multicenter collaborations in the context of international registry-based projects could promote data sharing, standardization of more clinical outcomes and knowledge diffusion that hopefully may optimize therapy and personalize the management of patients with such a complex syndrome.