What is the treatment approach for Bechets disease?

Behçet's Disease: Clinical Overview and Treatment Approach

Disease Characteristics

Behçet's disease is a chronic relapsing-remitting systemic vasculitis that affects vessels of all sizes, characterized by recurrent oral ulcers, genital ulcers, ocular inflammation, and skin lesions, with the primary treatment goal being prevention of irreversible organ damage through prompt immunosuppression tailored to specific organ involvement. 1, 2

Natural Course and Prognosis

• The disease follows an unpredictable relapsing-remitting pattern with inflammatory exacerbations that typically decrease in frequency and severity over time, often ameliorating as patients age beyond their forties 1, 3, 4
• Young males with early disease onset experience the most severe disease course and require aggressive immunosuppression with closer monitoring 2, 5, 4
• Mucocutaneous and joint manifestations impair quality of life but rarely cause permanent damage, whereas ocular, vascular, neurological, and gastrointestinal involvement can lead to irreversible organ damage and death if untreated 1, 2, 6

Organ-Specific Treatment Algorithm

Mucocutaneous Involvement

• Topical corticosteroids should be applied first-line for oral and genital ulcers 1
• Colchicine 1-2 mg/day is the first-line systemic therapy, particularly effective for erythema nodosum and genital ulcers, with proven efficacy in randomized controlled trials especially in women 1, 2
• Papulopustular or acne-like lesions are managed with topical or systemic acne treatments 1
• For refractory cases, escalate to azathioprine, thalidomide, interferon-alpha, TNF-alpha inhibitors, or apremilast 1

Arthritis

• Colchicine 1-2 mg/day is first-line treatment with demonstrated efficacy in randomized controlled trials 2

Ocular Involvement (Posterior Segment)

Any patient with posterior segment eye disease must receive mandatory combination therapy from the outset—never monotherapy 1, 2:

• Azathioprine combined with systemic corticosteroids is the mandatory first-line regimen 1, 2
• Alternative first-line options include cyclosporine-A, interferon-alpha, or monoclonal anti-TNF antibodies, all combined with corticosteroids 1, 2
• For acute sight-threatening uveitis, immediately initiate high-dose glucocorticoids with either infliximab or interferon-alpha 1
• Intravitreal glucocorticoid injection can be added for unilateral exacerbations as adjunct to systemic treatment 1

Isolated Anterior Uveitis

• Systemic immunosuppressives should be considered for patients with poor prognostic factors: young age, male sex, and early disease onset 1

Neurological Involvement

For acute parenchymal neurobehçet's disease, the treatment sequence is critical 2:

• Initiate high-dose intravenous methylprednisolone followed by oral prednisolone 2
• Combine with azathioprine 2.5 mg/kg/day from the outset as the preferred steroid-sparing agent 2
• TNF-alpha inhibitors, particularly infliximab, are recommended for refractory or severe cases failing conventional therapy 2
• CRITICAL CAVEAT: Never use cyclosporine-A in patients with CNS involvement due to significant neurotoxicity risk 2

Vascular Involvement

Acute Deep Vein Thrombosis

• Use immunosuppressive agents (corticosteroids combined with azathioprine, cyclophosphamide, or cyclosporine-A) as primary therapy—not anticoagulation 1, 2
• Anticoagulants, antiplatelet agents, or antifibrinolytic agents should not be used for venous thrombosis in Behçet's disease 2
• Anticoagulants may be added only in refractory cases if bleeding risk is low and pulmonary artery aneurysms are ruled out 1

Pulmonary Artery Aneurysms

• High-dose glucocorticoids combined with cyclophosphamide are the recommended regimen 1, 2
• Monoclonal anti-TNF antibodies should be considered for refractory cases 1
• For patients with high bleeding risk, embolization is preferred over open surgery 1

Peripheral and Aortic Artery Aneurysms

• Medical treatment with cyclophosphamide and corticosteroids must be initiated before any intervention 1, 2
• Surgery or stenting should not be delayed if the patient is symptomatic 1

Gastrointestinal Involvement

• Before considering surgery (except emergencies), trial the following sequentially: sulfasalazine, corticosteroids, azathioprine, TNF-alpha antagonists, or thalidomide 2
• Gastrointestinal involvement must be confirmed by endoscopy 1

Refractory Disease Management

• For patients failing conventional immunosuppression across any organ system, TNF-alpha inhibitors (infliximab or adalimumab) represent the next therapeutic step 2, 7
• Infliximab combined with azathioprine shows particular promise for severe or refractory cardiac involvement 2

Long-Term Management and Monitoring

• As disease manifestations typically ameliorate over time, treatment can be tapered and potentially discontinued in stable patients 1, 2, 3
• Regular monitoring of inflammatory markers and organ-specific assessments remains essential even during remission 2
• Patients with history of major organ involvement require continued monitoring despite symptom improvement 3

Critical Pitfalls to Avoid

• Never use cyclosporine-A in patients with CNS involvement due to neurotoxicity 2
• Never use anticoagulation as primary therapy for venous thrombosis—immunosuppression is the treatment 2
• Never use corticosteroid monotherapy for posterior uveitis—always combine with immunosuppressives 1
• Medication non-compliance can lead to irreversible organ damage despite the natural tendency for symptoms to improve 3
• Post-thrombotic syndrome from recurrent DVT can result in difficult-to-treat leg ulcers requiring vascular surgery consultation 1, 3