What is the initial treatment for Behcet disease?

Initial Treatment for Behçet Disease

The initial treatment for Behçet disease depends critically on organ involvement: colchicine (1-2 mg/day) is first-line for mucocutaneous lesions and arthritis, while sight-threatening posterior uveitis requires immediate combination therapy with azathioprine (2.5 mg/kg/day) plus systemic corticosteroids to prevent irreversible blindness. 1, 2

Treatment Algorithm Based on Organ Involvement

Mucocutaneous Disease (Most Common Presentation)

For isolated oral and genital ulcers:

• Start with topical corticosteroids as first-line therapy 1, 2
• These are appropriate for mild, localized lesions without systemic involvement 1

For recurrent mucocutaneous lesions:

• Colchicine 1-2 mg/day should be initiated, particularly when the dominant lesion is erythema nodosum or genital ulcers 1, 2
• This recommendation has Level IB evidence with Grade A strength, showing efficacy in two randomized controlled trials 1
• Colchicine is especially effective in women for preventing genital ulcers (P=0.004), erythema nodosum (P=0.004), and arthritis (P=0.033) 3
• Men also benefit, particularly for arthritis control (P=0.012) 3

For resistant mucocutaneous disease:

• Consider escalation to azathioprine, interferon-alpha, TNF-alpha antagonists, or apremilast 1, 2

Joint Involvement

Colchicine 1-2 mg/day is the initial treatment of choice for arthritis in Behçet disease 1

• This has Level IB evidence with Grade A recommendation 1
• Arthritis in Behçet disease typically follows a mild, transient course without deformities or erosions, mainly affecting large joints (knees, ankles) 1, 2
• Two RCTs demonstrated beneficial effects of colchicine for arthritis 1

Posterior Uveitis (Sight-Threatening Emergency)

Any patient with inflammatory eye disease affecting the posterior segment requires immediate combination therapy:

• Azathioprine 2.5 mg/kg/day PLUS systemic corticosteroids 1, 2
• This is a Level IB/IIA evidence recommendation with Grade A/B strength 1
• Never use systemic corticosteroids alone for posterior uveitis—they must be combined with azathioprine or other immunosuppressives 1, 2

For severe eye disease (>2 lines visual acuity drop or retinal vasculitis/macular involvement):

• Escalate to infliximab or cyclosporine A in combination with azathioprine and corticosteroids 1
• Alternative: interferon-alpha with or without corticosteroids 1
• Infliximab shows rapid response (1-5 days) for refractory disease 2

Acute Deep Vein Thrombosis

Immunosuppressive agents are recommended, NOT anticoagulation:

• Use glucocorticoids plus immunosuppressives (azathioprine, cyclophosphamide, or cyclosporine A) 1, 2
• This is Level III evidence with Grade C recommendation 1
• Anticoagulants are NOT recommended as venous thrombi in Behçet disease adhere to vessel walls and rarely embolize 1
• Critical pitfall: Avoid anticoagulation due to risk of fatal bleeding from coexistent pulmonary arterial aneurysms 1

Arterial Involvement

For pulmonary artery aneurysms:

• High-dose glucocorticoids plus cyclophosphamide are required 1, 2
• Consider monoclonal anti-TNF antibodies for refractory cases 1

Neurological Involvement

For parenchymal CNS disease:

• High-dose corticosteroids for acute attacks 4, 2
• Add immunosuppressives: azathioprine, interferon-alpha, cyclophosphamide, methotrexate, or TNF-alpha antagonists 1, 4

For dural sinus thrombosis:

• Corticosteroids alone are recommended 1, 2

Gastrointestinal Involvement

Medical therapy before surgery (except emergencies):

• Try sulfasalazine, corticosteroids, azathioprine, TNF-alpha antagonists, or thalidomide 1, 2
• This is Level III evidence with Grade C recommendation 1

Critical Pitfalls to Avoid

Cyclosporine A neurotoxicity:

• Do NOT use cyclosporine A in patients with CNS involvement unless absolutely necessary for intraocular inflammation 1, 4, 2
• Three case-control studies indicate potential neurotoxicity 1

Anticoagulation risks:

• Avoid anticoagulants in Behçet-related thrombosis due to bleeding risk, especially with potential pulmonary arterial aneurysms 1, 2
• Pulmonary embolism is rare despite high frequency of venous thrombosis 1

Corticosteroid monotherapy:

• Never use systemic corticosteroids alone for posterior uveitis—always combine with steroid-sparing immunosuppressives 1, 2

High-Risk Populations Requiring Aggressive Initial Treatment

Young men with early disease onset:

• Higher risk of severe disease, particularly neurological and ocular involvement 4, 2
• Consider early systemic immunosuppression with azathioprine 2

Evidence Quality Considerations

The 2018 EULAR update 1 represents the most recent and highest quality guideline, superseding the 2008 recommendations 1. The treatment approach is organ-specific rather than disease-specific, reflecting the heterogeneous nature of Behçet disease. The strongest evidence (Level IB, Grade A) supports colchicine for mucocutaneous/joint disease and azathioprine plus corticosteroids for posterior uveitis 1, 2.