From the Guidelines
The management of Bechet's syndrome requires a tailored approach based on disease severity and organ involvement, with the goal of promptly suppressing inflammatory exacerbations and recurrences to prevent irreversible organ damage, as recommended by the 2018 update of the EULAR recommendations for the management of Behçet's syndrome 1. The treatment approach should be individualized according to age, gender, type, and severity of organ involvement, as well as patient preferences.
- For mucocutaneous manifestations and arthritis, colchicine (0.5-1.2 mg daily) is recommended as the initial treatment, with azathioprine, interferon-alpha, or TNF-alpha inhibitors considered in recurrent and chronic cases 1.
- For oral and genital ulcers, topical corticosteroids (such as triamcinolone 0.1% paste or dexamethasone elixir) provide symptomatic relief.
- Systemic therapy often includes prednisone (0.5-1 mg/kg/day) for acute flares, with gradual tapering.
- For more severe disease, immunosuppressants such as azathioprine (2-3 mg/kg/day), cyclosporine (3-5 mg/kg/day), or mycophenolate mofetil (1-2 g/day) are necessary.
- TNF-alpha inhibitors like infliximab (5 mg/kg at weeks 0,2,6, then every 8 weeks) or adalimumab (40 mg every other week) are effective for refractory cases, particularly in patients with ocular involvement, as supported by evidence from prospective, non-comparative, open-label trials 1. Ocular involvement requires aggressive treatment, often combining corticosteroids with immunosuppressants to prevent vision loss, with a treatment regimen such as azathioprine, cyclosporine-A, interferon-alpha, or monoclonal anti-TNF antibodies 1. Vascular manifestations may require anticoagulation alongside immunosuppression. Regular monitoring for medication side effects and disease activity is essential, with treatment adjustments made based on clinical response, and patient education about avoiding triggers like stress and trauma is also important for comprehensive management.
From the Research
Management of Bechet's Syndrome
The management of Bechet's syndrome involves a range of pharmacological agents, including:
- Corticosteroids, which are the main treatment option in acute attacks of ocular Bechet's disease 2
- Immunomodulatory and immunosuppressive agents, such as colchicine, azathioprine, cyclosporine-A, interferon-alpha, and cyclophosphamide, which are used as steroid-sparing agents and to prevent further relapses 3
- Tumor necrosis factor alpha inhibitors, such as infliximab and adalimumab, which are increasingly used for various refractory Bechet's syndrome manifestations despite the lack of controlled studies 3
- Interferon alpha-2a, which has been shown to be effective in Bechet uveitis refractory to traditional treatment regimens and provides significant improvement in visual prognosis 2
Treatment Approaches
The treatment approach for Bechet's syndrome depends on the organs involved, the severity of the involvement, and prognostic factors 4. A treat-to-attack strategy would help improve long-term outcomes in Bechet's syndrome 4. The management aims to preserve function and quality of life and to avoid damage 4.
Pharmacological Agents
The choice of pharmacological agents depends on the clinical manifestations and disease severity 5. The following agents have been used in the treatment of Bechet's syndrome:
- Corticosteroids
- Colchicine
- Azathioprine
- Cyclosporine-A
- Interferon-alpha
- Cyclophosphamide
- Tumor necrosis factor alpha inhibitors, such as infliximab and adalimumab
- Interferon alpha-2a
Efficacy of Treatments
The efficacy of treatments for Bechet's syndrome has been evaluated in several studies. For example, a study found that interferon alpha-2a was effective in Bechet uveitis refractory to traditional treatment regimens and provided significant improvement in visual prognosis 2. Another study found that cyclosporine and azathioprine were effective in preventing eye involvement, and benzathine-penicillin was effective in preventing arthritis 6.