From the Guidelines
The treatment for Behçet's disease should be tailored to disease severity and organ involvement, with more aggressive therapy for neurological, vascular, or ocular manifestations, as recommended by the 2018 update of the EULAR recommendations for the management of Behçet's syndrome 1.
Key Treatment Considerations
- Corticosteroids like prednisone (typically 0.5-1 mg/kg/day) are often used for acute flares, while colchicine (0.6-1.2 mg daily) helps manage mucocutaneous symptoms.
- 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 daily) may be prescribed.
- TNF inhibitors like infliximab (5 mg/kg IV at 0,2, and 6 weeks, then every 8 weeks) or adalimumab (40 mg subcutaneously every 2 weeks) are effective for refractory cases, particularly with ocular involvement.
- Apremilast (30 mg twice daily) has shown benefit for oral and genital ulcers.
Organ-Specific Treatment
- Eye involvement: azathioprine, cyclosporine-A, interferon-alpha, or monoclonal anti-TNF antibodies, with systemic glucocorticoids used only in combination with azathioprine or other systemic immunosuppressives 1.
- Mucocutaneous involvement: topical measures, colchicine, azathioprine, thalidomide, interferon-alpha, or TNF-alpha inhibitors may be considered 1.
Monitoring and Adjustments
- Regular monitoring for medication side effects is essential.
- Treatment should be adjusted based on disease severity and organ involvement, with the goal of suppressing the abnormal inflammatory response that characterizes Behçet's disease.
From the Research
Treatment Options for Behçet's Disease
The treatment for Behçet's disease typically involves a combination of medications to manage symptoms and prevent complications. Some of the treatment options include:
- Glucocorticoids, which are used to induce remission 2, 3
- Azathioprine, which is used as a first-line conventional immunosuppressive in the maintenance phase to prevent relapses of major organ involvement 4, 2, 3
- Cyclosporine A, which is effective in patients with severe uveitis and extraocular manifestations 2
- Interferon alpha, which benefits ocular and extraocular manifestations 2, 3
- TNFα inhibitors, such as infliximab, which have been shown to be effective in treating resistant oral ulcers and other manifestations of Behçet's disease 5, 3
- Apremilast, which is approved by the FDA for oral ulcers 3
- IL-1 inhibitors, ustekinumab, secukinumab, and tocilizumab, which have shown promising results for refractory Behçet's disease 3
Specific Treatment Approaches
For patients with severe Behçet's disease, combination therapy with immunomodulatory agents and corticosteroids may be effective in preventing uveitis relapse 6. Adalimumab plus corticosteroids has been shown to be superior to ciclosporin plus corticosteroids in preventing uveitis relapse in patients with severe Behçet's disease naive to anti-TNF therapy 6.
Management of Specific Manifestations
The management of Behçet's disease involves treating specific manifestations, such as:
- Oral ulcers, which can be treated with topical steroids, local anesthetics, and systemic medications such as azathioprine and infliximab 5, 2
- Uveitis, which can be treated with cyclosporine A, azathioprine, and TNFα inhibitors 4, 6, 2
- Arthritis, which can be treated with colchicine and methotrexate 2
- Gastrointestinal vasculitis, which can be treated with sulfasalazine 2