From the Guidelines
The initial treatment approach for Behcet's disease focuses on symptom management based on disease severity and organ involvement, with topical corticosteroids and colchicine as first-line therapy for mild mucocutaneous manifestations, and systemic corticosteroids and immunosuppressive agents for more severe disease or organ involvement, as recommended by the 2018 update of the EULAR recommendations for the management of Behcet's syndrome 1.
Key Considerations
- For mild mucocutaneous manifestations, topical corticosteroids (such as triamcinolone 0.1% paste for oral ulcers or betamethasone 0.05% cream for genital ulcers) applied 2-3 times daily during flares are first-line therapy.
- Colchicine (0.5-1.2 mg daily) is often added as a systemic treatment to reduce the frequency and severity of mucocutaneous lesions.
- For more severe disease or organ involvement, systemic corticosteroids like prednisone (0.5-1 mg/kg/day) may be initiated with a gradual taper once symptoms improve.
- Immunosuppressive agents such as azathioprine (2-3 mg/kg/day), cyclosporine (3-5 mg/kg/day in divided doses), or TNF-alpha inhibitors like infliximab (5 mg/kg IV at weeks 0,2, and 6, then every 8 weeks) are used for refractory cases or major organ involvement.
Treatment Goals
- Prevent tissue damage and reduce morbidity by targeting the underlying immune dysregulation and excessive inflammatory response that characterizes Behcet's disease.
- Individualize treatment based on specific manifestations, with close monitoring for side effects and disease progression.
Relevant Guidelines
- The 2018 update of the EULAR recommendations for the management of Behcet's syndrome provides evidence-based guidance for the medical management of mucocutaneous, joint, eye, vascular, neurological, and gastrointestinal involvement of Behcet's disease 1.
From the Research
Initial Treatment Approach for Behcet's Disease
The initial treatment approach for patients presenting with symptoms of Behcet's disease typically involves a combination of medications to manage the various manifestations of the disease.
- Corticosteroids are often used as the primary treatment option for acute attacks of Behcet's disease, particularly for ocular and systemic involvement 2, 3.
- Colchicine may be useful for treating some of the manifestations of Behcet's syndrome, especially among women, and can help reduce the occurrence of genital ulcers, erythema nodosum, and arthritis 4.
- Interferon alpha-2a (IFN-α-2a) monotherapy has been shown to be effective in patients with Behcet uveitis refractory to conventional immunosuppressive agents, with significant improvement in visual prognosis 5.
- Tumor necrosis factor (TNF) inhibitors, such as infliximab, may be used for the treatment of resistant oral ulcers in Behcet's disease, and have been shown to result in complete remission and recovery of mouth aphtae within a short period of time 6.
Treatment Considerations
The choice of therapeutic agents for Behcet's disease is limited by the lack of clinical trials, and is often based on case reports, case series, and open-label clinical trials 3.
- Treatment should be individualized based on the involved organs, severity of the disease, and patient's background.
- The use of TNF inhibitors represents an important therapeutic advance for patients with severe and resistant disease, as well as for those with contraindications or intolerance to conventional treatments 3.