What is the best approach to manage a patient with Behcet disease, considering their symptoms and potential complications?

Management of Behçet Disease

Behçet disease requires organ-specific treatment stratification, with mucocutaneous and joint manifestations managed symptomatically while sight-threatening eye disease, vascular, neurological, or gastrointestinal involvement demands immediate aggressive immunosuppression to prevent irreversible organ damage and death. 1

Treatment Approach by Organ System

Mucocutaneous Disease

• Topical corticosteroids are first-line for isolated oral and genital ulcers, providing symptomatic relief without systemic exposure 2, 1
• Colchicine 1-2 mg/day is the preferred systemic agent, particularly effective for erythema nodosum and genital ulcers, with two randomized controlled trials demonstrating efficacy for arthritis 2, 1
• For acne-like lesions, topical measures similar to acne vulgaris treatment are sufficient as these are primarily cosmetic concerns 2
• Azathioprine, interferon-alpha, or TNF antagonists should be reserved for resistant mucocutaneous cases 2, 3

Joint Involvement

• Colchicine effectively manages arthritis in most Behçet patients, as the arthritis typically follows a mild, transient course without deformities or erosions 2
• Intra-articular corticosteroids are appropriate for acute monoarticular arthritis 1
• The large joints (knees, ankles) are predominantly affected, and erosive changes are rare 2

Ocular Disease (Sight-Threatening)

• Any patient with posterior segment inflammatory eye disease must receive azathioprine combined with systemic corticosteroids immediately 2
• For severe eye disease (≥2 lines visual acuity drop or retinal vasculitis/macular involvement), add either ciclosporine A or infliximab to the azathioprine-corticosteroid regimen, or alternatively use interferon-alpha with or without corticosteroids 2
• High-dose glucocorticoids with consideration of infliximab or interferon-alpha are recommended for acute sight-threatening exacerbations 1
• Three randomized controlled trials with ciclosporine A demonstrated rapid, significant improvement in visual acuity and reduced frequency/severity of ocular attacks, though renal dysfunction and hypertension are concerns 2

Vascular Disease

Deep Vein Thrombosis

• Immunosuppressive agents (corticosteroids, azathioprine, cyclophosphamide, or ciclosporine A) are mandatory for acute deep vein thrombosis, not anticoagulation 2, 1
• Anticoagulants, antiplatelet agents, and antifibrinolytic agents are NOT recommended for venous thrombosis management, as venous thrombi in Behçet disease adhere to vessel walls without causing emboli, and pulmonary embolism is rare despite high venous thrombosis frequency 2
• Monoclonal anti-TNF antibodies are recommended for refractory venous thrombosis 1

Arterial Aneurysms

• Cyclophosphamide combined with corticosteroids are recommended for pulmonary and peripheral arterial aneurysms 2
• Anticoagulation of pulmonary artery aneurysms is contraindicated due to fatal bleeding risk from coexisting aneurysms 2, 1

Neurological Involvement

Parenchymal Disease

• High-dose corticosteroids combined with azathioprine is the standard regimen for parenchymal CNS involvement 1
• Additional agents to consider include interferon-alpha, cyclophosphamide, methotrexate, and TNF antagonists 2
• Escalate to monoclonal anti-TNF antibodies for severe or refractory parenchymal disease 1

Dural Sinus Thrombosis

• Corticosteroids alone are recommended for dural sinus thrombosis presenting with increased intracranial pressure and headaches 2

Critical Contraindication

• Ciclosporine A must NOT be used in any Behçet patient with CNS involvement (even if inactive) unless absolutely necessary for intraocular inflammation due to neurotoxicity risk 2, 1

Gastrointestinal Involvement

• Trial sulfasalazine, corticosteroids, azathioprine, TNF antagonists, or thalidomide before considering surgery, except in emergencies such as perforation 2, 1
• Glucocorticoids provide rapid ulcer healing during acute exacerbations 1
• The deep penetrating ulcers (typically terminal ileum, ileocecal region, colon) have high perforation risk requiring emergency surgical intervention 2
• Azathioprine may decrease re-operation rates and should be used as maintenance therapy in surgical patients 2
• Monoclonal anti-TNF antibodies and/or thalidomide are recommended for severe or refractory gastrointestinal disease 1

Disease Course and Prognostic Factors

• Behçet disease follows a chronic relapsing-remitting course with exacerbations that typically decrease in frequency and severity over time, allowing treatment tapering or discontinuation as patients age 1, 4
• Young males with early disease onset have more severe disease courses requiring aggressive treatment and closer follow-up 1, 4, 3
• Mucocutaneous and joint involvement impair quality of life but do not cause permanent damage 1, 4
• Ocular, vascular, neurological, and gastrointestinal involvement are associated with poor prognosis and can cause serious damage or death if untreated 1, 4, 3

Critical Pitfalls to Avoid

• Delaying immunosuppression in major organ involvement results in permanent damage 1
• Using anticoagulation for Behçet-related thrombosis instead of immunosuppression is ineffective and potentially dangerous 2
• Administering ciclosporine A to patients with any neurological history (even remote) risks severe neurotoxicity 2, 1
• Undertreating chronic scarring ulcers can cause irreversible anatomical damage including oropharyngeal narrowing and obliterative genital scarring 1