From the Guidelines
Behçet's syndrome management requires a tailored approach based on organ involvement and disease severity, with the most recent and highest quality study recommending topical corticosteroids, colchicine, or apremilast as first-line treatments for mucocutaneous manifestations 1. For mucocutaneous manifestations, the following treatments are recommended:
- Topical corticosteroids for oral and genital ulcers
- Colchicine (0.5-1.2 mg daily) for the prevention of recurrent mucocutaneous lesions, especially when the dominant lesion is erythema nodosum or genital ulcer
- Apremilast (30 mg twice daily) as an alternative to colchicine
- Azathioprine, thalidomide, interferon-alpha, TNF-alpha inhibitors, or apremilast for selected cases with severe or recurrent mucocutaneous lesions 1 For joint involvement, colchicine should be the initial treatment, with azathioprine, interferon-alpha, or TNF-alpha inhibitors considered in recurrent and chronic cases 1. Vascular involvement, particularly deep vein thrombosis, requires immunosuppression with azathioprine or cyclophosphamide plus anticoagulation, while neurological Behçet's is treated with high-dose corticosteroids followed by cyclophosphamide or TNF inhibitors 1. Gastrointestinal involvement responds to 5-ASA compounds, corticosteroids, and immunosuppressants, and joint symptoms can be managed with colchicine or NSAIDs 1. Regular monitoring for disease activity and medication side effects is essential, with treatment aimed at suppressing the underlying inflammatory process that characterizes this multisystem vasculitis 1.
From the Research
Treatment Options for Behçet's Syndrome
- The cornerstone of therapy for inducing remission is systemic corticosteroids, whereas immunomodulatory and immunosuppressive agents such as colchicine, azathioprine, cyclosporine-A, interferon-alpha, and cyclophosphamide are used as steroid-sparing agents and to prevent relapses 2, 3.
- Tumor necrosis factor (TNF) alpha inhibitors (infliximab, adalimumab, etanercept) have been reported beneficial for aggressive, refractory or frequently relapsing cases 2, 3.
- Other treatment options include nonsteroidal anti-inflammatory drugs, methotrexate, sulfasalazine, and thalidomide 4, 5.
Management of Specific Manifestations
- Orogenital manifestations are treated with local application of corticosteroids or other medications 4.
- Ocular lesions can be treated with topical, paraocular and systemic corticosteroids, colchicine, dapsone, cyclosporine, azathioprine, methotrexate, cyclophosphamide and chlorambucil 5.
- Vascular, neurological, and gastrointestinal involvement are still problematic as there are no controlled studies for these manifestations 6.