What is the initial treatment for Behcet's disease?

Initial Treatment for Behçet's Disease

The initial 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 should be first-line treatment for isolated oral and genital ulcers 1
• Colchicine (1-2 mg/day) is effective for erythema nodosum and mild mucocutaneous lesions, particularly in women 1
• For resistant cases, consider azathioprine, interferon-alpha, or TNF-alpha antagonists 1, 2

Joint Involvement

• Colchicine (1-2 mg/day) is the initial treatment of choice for arthritis in Behçet's disease 1
• Arthritis in Behçet's disease typically follows a mild, transient course without deformities or erosions, mainly affecting large joints such as knees and ankles 1
• For recurrent and chronic cases, consider azathioprine, interferon-alpha, or TNF-alpha inhibitors 1

Eye Involvement

• Any patient with inflammatory eye disease affecting the posterior segment should receive azathioprine and systemic corticosteroids 1
• For severe eye disease (>2 lines drop in visual acuity or retinal disease), add either cyclosporine A or infliximab to the azathioprine and corticosteroids regimen 1
• Alternatively, interferon-alpha with or without corticosteroids can be used for severe eye disease 1, 3

Vascular Involvement

• For acute deep vein thrombosis, use immunosuppressive agents such as corticosteroids, azathioprine, cyclophosphamide, or cyclosporine A 1
• For pulmonary and peripheral arterial aneurysms, high-dose glucocorticoids and cyclophosphamide are recommended 1
• Anticoagulants are generally not recommended for venous thrombosis in Behçet's disease as the primary pathology is inflammation of the vessel wall rather than hypercoagulability 1

Neurological Involvement

• Acute attacks of parenchymal involvement should be treated with high-dose glucocorticoids followed by slow tapering, together with immunosuppressives such as azathioprine 1
• For cerebral venous thrombosis, high-dose glucocorticoids followed by tapering is recommended 1
• Cyclosporine A should be avoided in patients with central nervous system involvement due to potential neurotoxicity 1
• For severe or refractory neurological disease, consider TNF-alpha antagonists as first-line or in refractory patients 1

Gastrointestinal Involvement

• Medical treatment with sulfasalazine, corticosteroids, azathioprine should be tried before surgery, except in emergencies 1
• For severe/refractory cases, consider TNF-alpha antagonists and/or thalidomide 1
• Urgent surgical consultation is necessary for perforation, major bleeding, or obstruction 1

Common Pitfalls and Caveats

• Avoid cyclosporine A in patients with central nervous system involvement due to potential neurotoxicity 1
• Anticoagulants should generally be avoided in Behçet's disease with venous thrombosis due to risk of bleeding, especially with potential coexisting pulmonary arterial aneurysms 1
• Young men with early disease onset have higher risk of severe disease and may benefit from early systemic immunosuppression with azathioprine 1
• Post-thrombotic syndrome is frequent with recurrent deep vein thrombosis and may result in difficult-to-treat leg ulcers 1
• Regular monitoring for medication side effects is essential, particularly with immunosuppressive agents 4, 5

Treatment Algorithm

1. Assess disease severity and organ involvement
2. For mild disease (isolated mucocutaneous lesions, mild arthritis):
   • Start with colchicine 1-2 mg/day 1
   • Use topical corticosteroids for oral/genital ulcers 1
3. For moderate-severe disease or vital organ involvement:
   • Initiate systemic corticosteroids (prednisolone 0.5-1 mg/kg/day) 1
   • Add appropriate immunosuppressant based on organ involvement:
     • Azathioprine (2.5 mg/kg/day) for eye, neurological involvement 1
     • Cyclophosphamide for vascular involvement, especially arterial aneurysms 1
     • Interferon-alpha for refractory eye disease 3
4. For refractory disease:
   • Consider TNF-alpha inhibitors (infliximab, etanercept) 1, 2
   • Consider other targeted therapies based on specific organ involvement 5

Early aggressive treatment is crucial to prevent recurrences and irreversible damage to vital organs, particularly in patients with risk factors for severe disease 6.