What is the recommended treatment approach for a patient with suspected Behcet syndrome?

Treatment Approach for Behçet Syndrome

Treatment must be immediately stratified by organ involvement: mucocutaneous and joint disease requires symptom-based therapy, while sight-threatening eye disease, vascular, neurological, or gastrointestinal involvement demands urgent aggressive immunosuppression to prevent irreversible organ damage and death. 1, 2

Overarching Treatment Principles

The fundamental goal is to rapidly suppress inflammatory exacerbations and prevent relapses that cause permanent organ damage, with treatment intensity directly dependent on whether organ involvement threatens irreversible damage. 1, 2 Behçet syndrome follows a relapsing-remitting course with unpredictable exacerbations that typically decrease in frequency and severity over time. 2

A critical distinction exists between manifestations that impair quality of life versus those that cause permanent damage or death—this determines treatment aggressiveness. 2, 3

Mucocutaneous and Joint Involvement

For patients with isolated skin, mucosa, and joint disease without major organ involvement:

• Start with topical corticosteroids for symptomatic relief of oral and genital ulcers. 2
• Colchicine is first-line systemic therapy, particularly effective for erythema nodosum and genital ulcers. 2
• Use intra-articular corticosteroids for acute monoarticular arthritis. 2

These manifestations cause quality of life impairment but do not cause permanent damage, allowing treatment to be tailored according to symptom burden versus medication risks. 2

Ocular Involvement (Sight-Threatening)

For posterior uveitis or panuveitis—this is a medical emergency:

• Immediately initiate high-dose glucocorticoids at the first sign of posterior segment involvement. 2
• Consider infliximab or interferon-alpha for acute sight-threatening exacerbations from the outset. 2
• Regular ophthalmologic examinations are mandatory as recurrent sight-threatening eye disease is unpredictable and can occur despite aggressive immunosuppression. 4

Critical pitfall: Eye involvement is both a diagnostic criterion and a poor prognostic factor requiring immediate aggressive treatment. 5

Vascular Involvement

For acute deep vein thrombosis or other vascular manifestations:

• Mandatory treatment with glucocorticoids plus immunosuppressives immediately upon diagnosis. 2
• Use monoclonal anti-TNF antibodies for refractory venous thrombosis. 2

Critical pitfall: Do not anticoagulate pulmonary artery aneurysms due to bleeding risk. 2

Neurological Involvement (Neurobehçet)

For parenchymal central nervous system involvement:

• Standard regimen: high-dose glucocorticoids plus azathioprine (2.5 mg/kg/day) from the outset. 2, 6
• Escalate to monoclonal anti-TNF antibodies (particularly infliximab) for severe or refractory disease. 2, 6
• For acute attacks, use high-dose intravenous methylprednisolone followed by oral prednisolone with gradual tapering over 6-12 months. 6

Critical pitfall: Never use cyclosporine-A in patients with any history of neurological involvement, even if inactive, due to significant neurotoxicity risk. 2, 6

Gastrointestinal Involvement

For acute gastrointestinal exacerbations:

• Glucocorticoids for rapid ulcer healing plus disease-modifying agents such as 5-ASA or azathioprine. 2
• Monoclonal anti-TNF antibodies and/or thalidomide for severe or refractory disease. 2
• Confirm involvement through endoscopy and/or imaging, ruling out NSAID-induced ulcers, inflammatory bowel disease, and infections like tuberculosis. 5

Risk Stratification and Prognostic Factors

Young males with early disease onset have more severe disease courses, requiring more aggressive treatment and increased vigilance during follow-up. 2, 6 The presence of ocular, vascular, neurological, or gastrointestinal involvement is a poor prognostic factor demanding immediate aggressive immunosuppression. 2, 5

Treatment Duration and Tapering

As disease manifestations usually abate over time, treatment may be tapered and even stopped during the disease course in stable patients. 2, 6 However, delaying immunosuppression in major organ involvement can result in permanent damage—this is the most critical pitfall to avoid. 2

Multidisciplinary Approach

Management requires close collaboration between rheumatology, ophthalmology, neurology, gastroenterology, dermatology, and vascular surgery depending on organ involvement. 1, 6, 4 This multidisciplinary approach enables faster diagnosis and well-integrated treatment strategies. 3