What is the recommended treatment for Behcet's disease?

Treatment of Behçet's Disease

Treatment for Behçet's disease should be tailored to the specific 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

Treatment Based on Organ Involvement

Mucocutaneous Involvement

• Topical corticosteroids are first-line treatment for isolated oral and genital ulcers 2
• Colchicine (1-2 mg/day) should be tried first for prevention of recurrent mucocutaneous lesions, especially when the dominant lesion is erythema nodosum or genital ulcers 2, 1
• Colchicine is particularly effective in women for treating genital ulcers, erythema nodosum, and arthritis 3
• Papulopustular or acne-like lesions should be treated with topical or systemic measures as used in acne vulgaris 2
• For resistant cases, consider azathioprine, thalidomide, interferon-alpha, TNF-alpha inhibitors, or apremilast 2, 1
• Leg ulcers may have different causes (venous stasis or obliterative vasculitis) and treatment should be planned with dermatologist and vascular surgeon input 2

Eye Involvement

• Any patient with inflammatory eye disease affecting the posterior segment should be on a treatment regimen that includes azathioprine and systemic corticosteroids 2, 1
• Systemic glucocorticoids should be used only in combination with azathioprine or other systemic immunosuppressives 2
• For severe eye disease (>2 lines drop in visual acuity or retinal disease), add either cyclosporine-A, infliximab, or interferon-alpha to the azathioprine and corticosteroids regimen 2
• Patients with acute sight-threatening uveitis should receive high-dose glucocorticoids, infliximab, or interferon-alpha 2
• Intravitreal glucocorticoid injection can be considered for unilateral exacerbation as an adjunct to systemic treatment 2

Vascular Involvement

• For acute deep vein thrombosis, use glucocorticoids and immunosuppressives such as azathioprine, cyclophosphamide, or cyclosporine-A 2
• For refractory venous thrombosis, consider monoclonal anti-TNF antibodies 2
• Anticoagulants may be added for venous thrombosis only if the risk of bleeding is low and pulmonary artery aneurysms are ruled out 2, 1
• For pulmonary and peripheral arterial aneurysms, high-dose glucocorticoids and cyclophosphamide are recommended 2
• For aortic and peripheral artery aneurysms, medical treatment with cyclophosphamide and corticosteroids is necessary before surgical intervention 2

Neurological Involvement

• For parenchymal CNS involvement, use high-dose pulsed corticosteroids (3-7 pulses of IV methylprednisolone 1g/day) during attacks, followed by maintenance oral corticosteroids tapered over 2-3 months 2
• Immunosuppressives such as azathioprine, interferon-alpha, cyclophosphamide, methotrexate, or TNF-alpha antagonists should be given to prevent recurrences 2
• For dural sinus thrombosis, brief courses of corticosteroids are recommended 2
• Avoid cyclosporine-A in patients with central nervous system involvement due to its potential neurotoxicity 2, 1

Joint Involvement

• Colchicine (1-2 mg/day) is the treatment of choice for arthritis in Behçet's disease 2, 1
• Arthritis in Behçet's disease typically follows a mild, transient course without deformities or erosions 1

Gastrointestinal Involvement

• Medical treatment with sulfasalazine, corticosteroids, and azathioprine should be tried before surgery, except in emergencies 2, 1
• For severe/refractory cases, consider TNF-alpha antagonists and/or thalidomide 2, 1

Steroid Dosing Protocol

• 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 posterior segment eye inflammation: high-dose intravenous methylprednisolone pulses (1 g/day for 3-7 days), followed by oral prednisolone at 1 mg/kg/day 1
• Taper prednisolone by 5-10 mg every 10-15 days, aiming for maintenance dose of 5-10 mg/day 1
• Continue maintenance therapy for at least 2 years and for at least 12 months after normalization of inflammatory markers 1

Important Considerations and Pitfalls

• Young men with early disease onset have higher risk of severe disease and may benefit from early systemic immunosuppression 2, 1
• Disease manifestations typically ameliorate over time in many patients, allowing for treatment tapering and even discontinuation 2
• Always combine systemic corticosteroids with appropriate steroid-sparing agents to minimize long-term steroid exposure 1
• Post-thrombotic syndrome is frequent with recurrent deep vein thrombosis and may result in difficult-to-treat leg ulcers 1
• Combination therapy with colchicine and benzathine penicillin has shown better efficacy in controlling mucocutaneous manifestations than colchicine alone 4
• Interferon alfa-2b combined with colchicine and benzathine penicillin has demonstrated effectiveness in preventing eye involvement and extraocular complications 5