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, 2

• 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

Eye Involvement (Highest Priority - Prevents Blindness)

For posterior segment inflammatory eye disease:

• Immediately initiate azathioprine (2.5 mg/kg/day) PLUS systemic corticosteroids 1, 2
• This combination is mandatory as systemic corticosteroids should never be used alone in posterior uveitis 2
• Alternative options include cyclosporine-A, interferon-alpha, or monoclonal anti-TNF antibodies combined with corticosteroids 1

For severe/sight-threatening eye disease (>2 lines visual acuity drop or retinal vasculitis):

• Add either cyclosporine A or infliximab to the azathioprine-corticosteroid regimen 1, 2
• Alternatively, use interferon-alpha with or without corticosteroids 1
• High-dose intravenous methylprednisolone pulses (1g/day for 3-7 days) should be given for acute sight-threatening episodes, followed by oral prednisolone 1 mg/kg/day 2

Vascular Involvement

For acute deep vein thrombosis:

• Initiate immunosuppressive agents (glucocorticoids plus azathioprine, cyclophosphamide, or cyclosporine-A) 1, 2
• Anticoagulants are NOT recommended as venous thrombi in Behçet disease adhere to vessel walls and rarely embolize 1
• Critical caveat: Rule out coexisting pulmonary artery aneurysms before considering anticoagulation due to fatal bleeding risk 1

For pulmonary or peripheral arterial aneurysms:

• Start high-dose glucocorticoids plus cyclophosphamide immediately 1, 2
• Consider monoclonal anti-TNF antibodies for refractory cases 1

Neurological Involvement

For parenchymal CNS disease:

• Initiate high-dose corticosteroids for acute attacks 4, 2
• Add immunosuppressive agents: azathioprine, interferon-alpha, cyclophosphamide, methotrexate, or TNF-alpha antagonists 1, 4

For dural sinus thrombosis:

• Corticosteroids are recommended as primary therapy 1

Critical warning: Cyclosporine A should NOT be used in patients with CNS involvement due to potential neurotoxicity, unless absolutely necessary for intraocular inflammation 1, 4, 2

Gastrointestinal Involvement

Initial medical therapy before surgery:

• Try sulfasalazine, corticosteroids, azathioprine, TNF-alpha antagonists, or thalidomide 1, 2
• Surgery is reserved for emergencies (perforation) 1
• No evidence-based first-line treatment exists; choice depends on severity 1

Critical Pitfalls to Avoid

• Never use systemic corticosteroids alone for posterior uveitis - always combine with azathioprine or other immunosuppressives 2
• Avoid cyclosporine A in any patient with neurological symptoms due to neurotoxicity risk 1, 4, 2
• Do not routinely anticoagulate venous thrombosis - use immunosuppression instead, as thrombi are inflammatory and adherent 1
• Screen for pulmonary artery aneurysms before any anticoagulation to prevent fatal hemorrhage 1
• Young men with early disease onset require aggressive early treatment as they have higher risk of severe organ involvement 4, 2

Corticosteroid Dosing When Indicated

For acute severe manifestations requiring systemic steroids:

• Start with oral prednisolone 1 mg/kg/day for acute attacks 2
• For sight-threatening uveitis or severe vascular disease: IV methylprednisolone 1g/day for 3-7 days, then oral prednisolone 1 mg/kg/day 1, 2
• Taper gradually over 2-3 months based on clinical response 1, 2
• Always combine with steroid-sparing immunosuppressives (azathioprine 2.5 mg/kg/day preferred) 2