What is the latest management approach for Behçet's disease?

Latest Management Approach for Behçet's Disease

The management of Behçet's disease should be tailored according to organ involvement, with colchicine as first-line therapy for mild mucocutaneous and joint manifestations, while systemic corticosteroids combined with immunosuppressants such as azathioprine are recommended for more severe organ involvement. 1

General Principles

• Behçet's disease typically follows a relapsing and remitting course, with the primary goal of treatment being to promptly suppress inflammatory exacerbations to prevent irreversible organ damage 2, 3
• Disease manifestations often ameliorate over time in many patients, potentially allowing for treatment tapering 3
• Ocular, vascular, neurological, and gastrointestinal involvement are associated with poor prognosis and require more aggressive treatment 2, 3

Treatment by Organ System

Mucocutaneous Involvement

• Topical corticosteroids are first-line treatment for isolated oral and genital ulcers 2, 1
• Colchicine (1-2 mg/day) should be tried first for prevention of recurrent mucocutaneous lesions, especially for erythema nodosum and genital ulcers 2, 1
• Papulopustular or acne-like lesions should be treated with topical or systemic measures as used in acne vulgaris 2
• For refractory cases, consider azathioprine, thalidomide, interferon-alpha, TNF-alpha inhibitors, or apremilast 2, 1
• Leg ulcers require collaborative management with dermatology and vascular surgery, as they may result from venous stasis or obliterative vasculitis 2

Eye Involvement

• Management of uveitis requires close collaboration with ophthalmologists 2
• Any patient with inflammatory eye disease affecting the posterior segment should receive azathioprine, cyclosporine-A, interferon-alpha, or monoclonal anti-TNF antibodies 2, 1
• Systemic glucocorticoids should only be used in combination with immunosuppressives, never as monotherapy 2, 1
• For acute sight-threatening uveitis, use high-dose glucocorticoids, infliximab, or interferon-alpha 2, 1
• Intravitreal glucocorticoid injection can be considered for unilateral exacerbation as an adjunct to systemic treatment 2
• For isolated anterior uveitis, consider systemic immunosuppressives in patients with poor prognostic factors (young age, male sex, early disease onset) 2

Vascular Involvement

• For acute deep vein thrombosis, use glucocorticoids and immunosuppressives such as azathioprine, cyclophosphamide, or cyclosporine-A 2
• Monoclonal anti-TNF antibodies should be considered for refractory venous thrombosis 2
• Anticoagulants are generally not recommended but may be added if bleeding risk is low and pulmonary artery aneurysms have been ruled out 2
• For pulmonary artery aneurysms, high-dose glucocorticoids and cyclophosphamide are recommended, with anti-TNF antibodies for refractory cases 2, 1
• For aortic and peripheral artery aneurysms, use cyclophosphamide and corticosteroids before surgical intervention 2
• Embolization is preferred to open surgery for patients at high risk of major bleeding 2

Neurological Involvement

• For parenchymal CNS involvement, high-dose glucocorticoids followed by immunosuppressives such as azathioprine, interferon-alpha, cyclophosphamide, methotrexate, or TNF-alpha antagonists 2, 1, 4
• For dural sinus thrombosis, high-dose glucocorticoids are recommended 2, 1
• Avoid cyclosporine A in patients with CNS involvement due to potential neurotoxicity 2, 3
• For chronic progressive neurological disease, methotrexate may be beneficial, while refractory cases may respond to infliximab 4

Gastrointestinal Involvement

• Medical treatment should be tried before surgery except in emergencies 2, 1
• Options include sulfasalazine, corticosteroids, azathioprine, TNF-alpha antagonists, or thalidomide 2, 1
• Gastrointestinal involvement is characterized by deep penetrating ulcers that may perforate, requiring careful monitoring 2

Joint Involvement

• Colchicine (1-2 mg/day) is usually effective for arthritis, which typically follows a mild and transient course without deformities or erosions 2, 1
• Alternative options include indomethacin and benzathine penicillin for refractory cases 2

Corticosteroid Regimen

• For acute attacks requiring systemic therapy, start with oral prednisolone at 1 mg/kg/day followed by gradual taper over 2-3 months 1
• For severe manifestations (posterior uveitis, CNS involvement, major vessel disease), consider intravenous methylprednisolone pulses (1 g/day for 3-7 days) 1, 4
• Always combine systemic corticosteroids with appropriate steroid-sparing agents 1
• After clinical response, taper prednisolone by 5-10 mg every 10-15 days, aiming for a maintenance dose of 5-10 mg/day 1

Important Considerations

• Young men with early disease onset have higher risk of severe disease and may benefit from early aggressive immunosuppression 1, 3
• Monitor for steroid-related side effects and provide calcium and vitamin D supplementation 1
• Regular assessment of clinical symptoms, inflammatory markers, and organ-specific monitoring is essential 1
• If inadequate response after 3 days of high-dose steroids for severe manifestations, consider early introduction of biologics 1

Emerging Therapies

• TNF-alpha inhibitors, particularly infliximab, have shown efficacy for refractory manifestations including uveitis, neurological, and vascular involvement 5, 6, 4
• Other biologics being investigated include tocilizumab, IL-1 inhibitors, and B-cell depletion therapy for severe and multidrug-resistant cases 4