What are the treatment recommendations for Behçet’s disease in a young adult male, including first‑line therapy for mild mucocutaneous disease and escalation options for ocular, vascular, or neurologic involvement?

Treatment of Behçet's Disease in Young Adult Males

For a young adult male with Behçet's disease, treatment must be stratified by organ involvement: use colchicine and topical corticosteroids for mild mucocutaneous disease, but immediately escalate to high-dose glucocorticoids plus azathioprine (or infliximab for severe cases) for any sight-threatening ocular, vascular, or neurologic involvement to prevent irreversible organ damage. 1, 2

Risk Stratification for Young Males

Young men with early disease onset represent a high-risk population requiring heightened vigilance and more aggressive treatment:

• Young males experience more severe disease courses with higher rates of major organ involvement, particularly ocular, vascular, and neurologic manifestations 1, 2, 3
• Systemic immunosuppression with azathioprine should be considered even for isolated anterior uveitis in young males with early disease onset, as they have higher risk of progression to posterior uveitis 1
• This population requires closer monitoring and lower threshold for treatment escalation 2, 4

First-Line Therapy for Mild Mucocutaneous Disease

For oral/genital ulcers, skin lesions, and arthritis without major organ involvement:

• Topical corticosteroids provide symptomatic relief for oral and genital ulcers 2
• Colchicine is the first-line systemic therapy, particularly effective for erythema nodosum and genital ulcers 2
• Intra-articular corticosteroids for acute monoarticular arthritis 2
• These manifestations impair quality of life but do not cause permanent organ damage, allowing treatment decisions to balance symptom control against medication risks 5, 2

Escalation for Ocular Involvement

Posterior uveitis and retinal vasculitis are sight-threatening emergencies:

• Any patient with posterior segment involvement requires azathioprine 2.5 mg/kg/day plus systemic corticosteroids immediately 1
• Azathioprine prevents hypopyon uveitis attacks (NNT=4), stabilizes visual acuity, and prevents new eye disease development (NNT=2) 1
• For severe eye disease (>2 lines visual acuity drop or retinal vasculitis), add either infliximab or interferon-alpha to the azathioprine and corticosteroid regimen 1, 2
• Alternatively, ciclosporine A 2-5 mg/kg/day can be added for refractory disease 1
• Local corticosteroid injections may be used as adjunct only, never as monotherapy 1

Escalation for Vascular Involvement

Acute Deep Vein Thrombosis

• Mandatory treatment with glucocorticoids plus immunosuppressives (azathioprine, cyclophosphamide, or cyclosporine-A) 1, 2
• The pathology is vessel wall inflammation, not hypercoagulability, making immunosuppression the cornerstone of treatment 1
• Anticoagulants may be added if bleeding risk is low, though evidence shows they do not decrease relapse risk 1
• Post-thrombotic syndrome is common with recurrent episodes and can cause difficult-to-treat leg ulcers 1, 4

Refractory Venous Thrombosis

• Monoclonal anti-TNF antibodies (infliximab or adalimumab) should be used for patients not responding to standard immunosuppression 1, 2
• Interferon-alpha may be tried in selected cases 1

Pulmonary Artery Aneurysms

• High-dose glucocorticoids (three successive IV methylprednisolone pulses followed by oral prednisolone 1 mg/kg/day) plus cyclophosphamide (monthly IV pulses) 1
• Infliximab for refractory cases 1
• Never anticoagulate pulmonary artery aneurysms due to bleeding risk 2
• Surgery only for life-threatening situations due to high mortality 1

Escalation for Neurologic Involvement

Parenchymal Involvement

• High-dose glucocorticoids (daily IV methylprednisolone 1g for up to 7 days, then oral prednisolone 1 mg/kg/day for 1 month with slow taper) plus azathioprine 1, 2
• Monoclonal anti-TNF antibodies should be considered for severe disease at onset or refractory/relapsing disease despite corticosteroids and azathioprine 1, 2
• Tocilizumab has shown benefit in limited observations 1

Cerebral Venous Thrombosis

• High-dose glucocorticoids followed by tapering 1
• Anticoagulants may be added for short duration 1
• Screen for extracranial vascular disease 1

Critical Pitfalls to Avoid

• Never use cyclosporine-A in patients with any history of neurologic involvement, even if currently inactive, as it may worsen neurologic disease 1, 2
• Do not delay immunosuppression in major organ involvement—irreversible damage occurs rapidly in ocular, vascular, and neurologic disease 5, 2
• Do not rely on anticoagulation alone for venous thrombosis—the primary pathology is vessel wall inflammation requiring immunosuppression 1
• Avoid surgery for pulmonary artery aneurysms except in life-threatening situations due to high surgical mortality 1
• Undertreating chronic ulcers can cause irreversible anatomical damage including oropharyngeal narrowing and obliterative genital scarring 2

Disease Course and Treatment Tapering

• Behçet's disease follows a chronic relapsing-remitting course with exacerbations that typically decrease in frequency and severity over time 5, 2, 4
• As disease manifestations abate, treatment can be tapered and potentially discontinued 2, 4
• However, patients with history of major organ involvement require continued monitoring even during remission 4