Life Expectancy and Quality of Life in Behçet's Disease vs. Neuro-Behçet's Disease
Patients with Behçet's disease generally have a good life expectancy with proper treatment, while those with neuro-Behçet's disease have a significantly worse prognosis with 25% becoming dependent or dying after approximately 6 years of follow-up.
General Prognosis and Life Expectancy
- Behçet's disease (BD) typically follows a relapsing and remitting course, with disease manifestations that often ameliorate over time in many patients 1
- Ocular, vascular, neurological, and gastrointestinal involvement are associated with poor prognosis and can cause serious damage or death if left untreated 1
- When only skin, mucosa, and joint involvement are present, the prognosis is generally good and does not cause permanent damage 1
- Young men with early disease onset have a more severe disease course, requiring more aggressive treatment and careful follow-up 1
Neuro-Behçet's Disease (NBD) Prognosis
- After a median follow-up of 73 months (approximately 6 years), 25.2% of patients with NBD become dependent (unable to perform activities of daily living) or die 2
- The 5-year and 7-year event-free survival rates for NBD are 65% and 53%, respectively 2
- Factors independently associated with poor outcome in NBD include:
Quality of Life Impact
- BD significantly impairs quality of life compared to healthy individuals 3
- Physical health scores and several domains of quality of life (Role Physical, Bodily Pain, General Health, Vitality, and Mental Health) are significantly lower in BD patients 3
- Quality of life impairment correlates with disease activity and specific clinical manifestations 3
- The presence of genital ulcers, eye involvement, and central nervous system (CNS) involvement are particularly associated with reduced quality of life 3
Types of Neurological Involvement
- Neurological involvement in BD (neuro-Behçet's disease) occurs in approximately 5-10% of all BD cases 4
- NBD can be classified into two major forms:
- Parenchymal NBD (majority of cases): characterized by focal or multifocal CNS involvement, often presenting as subacute brainstem syndrome and hemiparesis 4
- Extra-axial NBD (10-20% of cases): caused by isolated cerebral venous sinus thrombosis and intracranial hypertension, with fewer symptoms and better neurological prognosis 4
- NBD usually develops 1-10 years after the first symptom of BD 5
Treatment Impact on Prognosis
- Early aggressive intervention with corticosteroids and cytotoxic agents can significantly improve the prognosis in NBD patients 5
- In severe NBD cases (Rankin score ≥3 at onset), intravenous cyclophosphamide appears to provide longer event-free survival compared to azathioprine 2
- For parenchymal NBD, treatment options include high-dose intravenous methylprednisolone followed by oral tapering, along with immunosuppressants such as azathioprine, cyclophosphamide, interferon-alpha, or TNF-alpha inhibitors 6, 4
- Cyclosporine A should be avoided in patients with CNS involvement due to potential neurotoxicity 7
Biomarkers and Monitoring
- CSF interleukin-6 levels correlate with NBD activity, therapeutic responses, and prognosis 8
- Regular monitoring of disease activity through clinical symptoms and inflammatory markers is essential 7
- For patients with ocular involvement, regular ophthalmologic examinations are crucial 7
Common Pitfalls in Management
- Untreated NBD can lead to severe disability or death, highlighting the importance of prompt diagnosis and treatment 5
- Young men with early disease onset have a higher risk of severe disease and may benefit from early systemic immunosuppression 7
- Post-thrombotic syndrome is frequent with recurrent deep vein thrombosis and may result in difficult-to-treat leg ulcers 7