From the Guidelines
Treatment for neurobehçet's disease typically follows a step-wise approach, starting with high-dose corticosteroids as the first-line therapy, as recommended by the 2018 update of the EULAR recommendations for the management of Behçet's syndrome 1.
First-Line Therapy
Begin with intravenous methylprednisolone 1 gram daily for 3-5 days, followed by oral prednisone 1 mg/kg/day. Gradually taper the prednisone dose over several months based on clinical response. Concurrently, initiate an immunosuppressive agent such as azathioprine (2.5 mg/kg/day) or mycophenolate mofetil (2-3 g/day in divided doses). These drugs help maintain remission and allow for steroid tapering.
Second-Line Therapy
For more severe cases or those refractory to initial treatment, consider cyclophosphamide (750-1000 mg/m² IV monthly) or infliximab (5 mg/kg IV at 0,2, and 6 weeks, then every 8 weeks), as supported by the guidance on noncorticosteroid systemic immunomodulatory therapy in noninfectious uveitis 1. In cases of venous sinus thrombosis, add anticoagulation therapy with low-molecular-weight heparin followed by warfarin, aiming for an INR of 2-3.
Monitoring and Maintenance
Monitor patients closely for treatment response and side effects. Regular blood tests, including complete blood count and liver function tests, are essential. Continue treatment for at least 2 years after achieving remission. The goal is to suppress inflammation and prevent recurrence of neurological symptoms. This aggressive approach is necessary due to the potential for severe and irreversible neurological damage in neurobehçet's disease, as highlighted by the EULAR recommendations 1 and other studies 1.
Some key points to consider:
- The use of adalimumab for the treatment of NIU is supported (EL 1B) 1.
- The use of infliximab for the treatment of NIU is supported (EL 2B) 1.
- Interferon alfa-2a in non-anterior NIU is supported (EL 2B) 1.
- There is no evidence to support the use of etanercept in NIU (EL 2B) 1.
- The use of subcutaneous secukinumab in non-anterior NIU is not supported (EL 2B) 1.
From the Research
Treatment Lines for Neuro-Behçet's Disease
The treatment of Neuro-Behçet's disease can be divided into several lines, including:
- First-line treatment: azathioprine or methotrexate and corticosteroids for patients with parenchymal neuro-Behçet's disease without poor prognostic factors 2
- High-risk patients: intravenous cyclophosphamide and corticosteroids 2
- Treatment of acute attacks: high-dose intravenous corticosteroids followed by maintenance treatment with oral steroids for 6-12 months 3
- Prevention of relapses: oral immunosuppressants such as azathioprine and mycophenolate 3
- Refractory patients: cyclophosphamide, interferon alpha, or anti-TNF-α monoclonal antibodies such as infliximab, etanercept, and adalimumab 3
- Newer agents: tocilizumab, canakinumab, and anakinra, which exert their biological activity through IL-1 and IL-6 pathways 3
- Repeated low-dose rituximab treatment: effective in relapsing neuro Behçet's disease 4
- Infliximab treatment: a good candidate for neuro-Behçet's disease treatment when it is resistant to conventional immunosuppressive agents 5
Specific Treatment Approaches
- Parenchymal NBD: glucocorticoids alone or in combination with azathioprine, with more potent immunosuppressive drugs such as mycophenolate, methotrexate, cyclophosphamide, or targeted therapy for relapsing-remitting or progressive courses 6
- CVST: immunosuppressive drugs with or without anticoagulation 6
- Venous sinus thrombosis: anticoagulation and short-term corticosteroids with or without immunosuppressants 2