Likelihood of Developing Neuro-Behçet's in Patients with Behçet's Disease and Retinal Vasculitis
Patients with Behçet's disease who have retinal vasculitis are at significant risk for developing neurological manifestations, with cerebral venous sinus thrombosis being a particularly common neurological complication in this population. 1
Risk Factors and Disease Progression
- Young men with early disease onset have a higher risk of severe disease progression, including neurological involvement, and require more aggressive treatment and closer follow-up 2, 3
- Retinal vasculitis represents posterior segment involvement, which is considered a marker of more severe disease that may predispose to other major organ involvement, including neurological manifestations 4, 1
- Untreated eye, vascular, and neurological system involvement can cause serious damage and even death, highlighting the connection between these manifestations 4
Clinical Considerations
- Behçet's disease follows a relapsing and remitting course, with inflammatory exacerbations that generally decrease in frequency and severity over time 2
- Posterior uveitis and retinal vasculitis are the main causes of severe vision loss or blindness in patients with Behçet's disease, indicating the severity of this manifestation 5
- The presence of retinal vasculitis often signals a more aggressive disease phenotype that may involve multiple organ systems 6
Management Implications
- Any patient with Behçet's disease and inflammatory eye disease affecting the posterior segment (including retinal vasculitis) should be on a treatment regimen such as azathioprine, cyclosporine-A, interferon-alpha, or monoclonal anti-TNF antibodies 4
- For patients with retinal vasculitis, systemic immunosuppressives are mandatory as glucocorticoids should never be used alone in patients with posterior uveitis 4
- Patients with retinal vasculitis should be monitored closely for signs of neurological involvement, particularly cerebral venous sinus thrombosis 1
- Cyclosporine A should be avoided in Behçet's patients with central nervous system involvement unless necessary for intraocular inflammation, due to potential neurotoxicity 1, 3
Treatment Approach for Patients with Retinal Vasculitis
- First-line treatment for retinal vasculitis includes azathioprine combined with systemic corticosteroids 1, 7
- For severe eye disease, either cyclosporine A or infliximab in combination with azathioprine and corticosteroids is recommended 1
- A combination of cyclophosphamide with prednisolone or cyclosporin with prednisolone has shown effectiveness for long-term control of retinal vasculitis in Behçet's disease 5
- If neurological involvement develops, cyclophosphamide and methotrexate are recommended for managing acute phase neurological involvement 1
Monitoring and Prevention
- Regular ophthalmologic examinations are essential for patients with ocular involvement to monitor disease activity and detect early signs of complications 1
- Monitoring should include assessment of clinical symptoms and inflammatory markers regularly, with consideration of repeat imaging for suspected neurological involvement 1
- Early and intensive treatment is warranted in patients with retinal vasculitis to prevent irreversible damage to both ocular and neurological tissues 7