Management of Behçet Syndrome

Overview of Treatment Principles

The European League Against Rheumatism recommends that Behçet syndrome management must be stratified by organ involvement, with mucocutaneous/joint disease requiring symptom-based treatment and sight-threatening eye disease, vascular, neurological, or gastrointestinal involvement demanding immediate aggressive immunosuppression to prevent irreversible organ damage and death 1, 2
The goal of treatment is to rapidly suppress inflammatory exacerbations and prevent relapses that cause irreversible organ damage, with treatment intensity depending on whether organ involvement threatens permanent damage 1, 2

The European League Against Rheumatism suggests using topical corticosteroids for symptomatic relief of oral and genital ulcers 1, 2
Colchicine is recommended as first-line systemic therapy, especially effective for erythema nodosum and genital ulcers 1, 2
Intra-articular corticosteroids are recommended for acute monoarticular arthritis 3

For posterior uveitis or panuveitis, the American College of Rheumatology recommends starting immediately with high-dose glucocorticoids and considering infliximab or interferon-alpha for acute sight-threatening exacerbations 3, 4

For acute deep vein thrombosis, the European League Against Rheumatism recommends mandatory treatment with glucocorticoids and immunosuppressives 3, 4
Monoclonal anti-TNF antibodies are recommended for refractory venous thrombosis 3

For parenchymal involvement, the American College of Rheumatology recommends a standard regimen of high-dose glucocorticoids and azathioprine, with escalation to monoclonal anti-TNF antibodies for severe or refractory disease 3, 4

For acute exacerbations, the European League Against Rheumatism recommends glucocorticoids for rapid ulcer healing and disease-modifying agents such as 5-ASA or azathioprine 3, 4
Monoclonal anti-TNF antibodies and/or thalidomide are recommended for severe or refractory disease 3, 4

The American College of Rheumatology advises against using cyclosporine-A in patients with any history of neurological involvement, even if inactive 3, 4
Anticoagulation of pulmonary artery aneurysms is not recommended due to bleeding risk 3
Delaying immunosuppression in major organ involvement can result in permanent damage, and undertreating chronic scarring ulcers can cause irreversible anatomical damage 1, 2

The European League Against Rheumatism recommends that Behçet's disease is characterized by a chronic relapsing-remitting course with unpredictable exacerbations that typically decrease in frequency and severity over time, with disease manifestations often ameliorating in many patients as they age 5, 6

The American College of Rheumatology states that genital ulcers develop in the majority of patients and can cause scarring if untreated, potentially leading to oropharyngeal narrowing and obliterative/deforming genital scarring 5, 6
The European League Against Rheumatism notes that skin lesions (erythema nodosum, papulopustular lesions) fluctuate with disease activity 5
The American College of Rheumatology recommends that these manifestations cause impairment of quality of life but do not cause permanent damage 6
Ocular, vascular, neurological, and gastrointestinal involvement are associated with poor prognosis and can cause serious damage and even death if untreated, according to the European League Against Rheumatism 5, 6, 7

The American College of Rheumatology states that young males with early disease onset have a more severe disease course, prompting more aggressive treatment and increased caution during follow-up 5, 6, 7

The European League Against Rheumatism recommends that when only skin, mucosa, and joint involvement exists, treatment can be tailored according to quality of life impact versus medication risks 5, 6
The American College of Rheumatology notes that when major organ involvement occurs, rapid suppression of inflammation and prevention of relapses is critical to prevent loss of function, usually requiring immunosuppressives 5, 6
The European League Against Rheumatism states that as disease manifestations usually abate over time, treatment may be tapered and even stopped during the disease course 5, 6