What is the initial treatment for Behcet's syndrome?

Initial Treatment for Behçet's Syndrome

The initial treatment for Behçet's syndrome 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 used as first-line treatment for isolated oral and genital ulcers 1
• Colchicine (1-2 mg/day) is the first systemic treatment for recurrent mucocutaneous lesions, particularly effective for erythema nodosum and genital ulcers 2, 1
• For papulopustular or acne-like lesions, topical or systemic measures as used in acne vulgaris should be employed 2
• For refractory cases, consider azathioprine, thalidomide, interferon-alpha, or TNF-alpha inhibitors 2, 1

Joint Involvement

• Colchicine should be the initial treatment for acute arthritis in Behçet's syndrome 2
• Acute monoarticular disease can be treated with intra-articular glucocorticoids 2
• For recurrent and chronic arthritis, consider azathioprine, interferon-alpha, or TNF-alpha inhibitors 2, 1

Eye Involvement

• Any patient with inflammatory eye disease affecting the posterior segment should be on azathioprine, cyclosporine-A, interferon-alpha, or monoclonal anti-TNF antibodies 2
• Systemic glucocorticoids should only be used in combination with immunosuppressives, not as monotherapy 2
• For acute sight-threatening uveitis, high-dose glucocorticoids, infliximab, or interferon-alpha should be initiated promptly 2, 1
• Intravitreal glucocorticoid injection can be used as an adjunct to systemic treatment for unilateral exacerbations 2

Vascular Involvement

• For acute deep vein thrombosis, glucocorticoids and immunosuppressives such as azathioprine, cyclophosphamide, or cyclosporine-A are recommended 2, 1
• For pulmonary artery aneurysms, high-dose glucocorticoids and cyclophosphamide are the initial treatment of choice 2
• For refractory vascular disease, monoclonal anti-TNF antibodies should be considered 2
• Anticoagulants should generally be avoided in Behçet's with venous thrombosis due to risk of bleeding, especially with potential coexisting pulmonary arterial aneurysms 1

Neurological Involvement

• Acute attacks of parenchymal involvement should be treated with high-dose glucocorticoids followed by slow tapering, together with azathioprine 2, 1
• For severe or refractory neurological disease, monoclonal anti-TNF antibodies should be considered as first-line or in refractory cases 2
• Cyclosporine-A should be avoided in patients with neurological involvement due to potential neurotoxicity 2, 1
• For cerebral venous thrombosis, high-dose glucocorticoids followed by tapering is recommended, with anticoagulants possibly added for a short duration 2

Gastrointestinal Involvement

• Gastrointestinal involvement should be confirmed by endoscopy and/or imaging, ruling out NSAID ulcers, inflammatory bowel disease, and infections 2
• For acute exacerbations, glucocorticoids should be used together with disease-modifying agents such as 5-ASA or azathioprine 2, 1
• For severe/refractory cases, monoclonal anti-TNF antibodies and/or thalidomide should be considered 2, 1
• Urgent surgical consultation is necessary in cases of perforation, major bleeding, and obstruction 2

Practical Approach to Initial Treatment

1. Assess disease severity and organ involvement 1

2. For mild disease (isolated mucocutaneous or joint involvement):

   • Start with colchicine 1-2 mg/day 2, 1
   • Use topical corticosteroids for acute ulcerations 2

3. For moderate to severe disease (major organ involvement):

   • Begin with systemic corticosteroids (typically prednisone 1 mg/kg/day) 2, 3
   • Add an immunosuppressive agent based on organ involvement:
     • Eye: Azathioprine or cyclosporine-A 2, 1
     • Vascular: Cyclophosphamide or azathioprine 2
     • Neurological: Azathioprine (avoid cyclosporine-A) 2, 1
     • Gastrointestinal: 5-ASA or azathioprine 2, 1

Common Pitfalls and Caveats

• Young men with early disease onset have a 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
• Corticosteroids should not be used alone for posterior uveitis but always in combination with immunosuppressives 2
• The vasculitis in Behçet's is primarily inflammatory rather than hypercoagulable, so immunosuppression rather than anticoagulation should be the focus of treatment 2, 1
• Regular monitoring for medication side effects is essential, particularly for cyclosporine nephrotoxicity, azathioprine myelosuppression, and cyclophosphamide-induced bladder toxicity 4, 5