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Behçet's syndrome

Background

Overview

Definition
BS is a chronic, relapsing autoimmune disorder characterized by systemic inflammation that primarily affects blood vessels throughout the body.
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Pathophysiology
The pathophysiology of BS involves dysregulation of the immune system, leading to systemic inflammation. This inflammation primarily affects the blood vessels, resulting in vasculitis, which can occur in both the venous and arterial systems.
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Epidemiology
The prevalence of BS in Northern Europe and the US ranges between 0.12 and 0.64 cases per 100,000 population. The highest prevalence of BS is reported in Turkey, with 420 cases per 100,000 population.
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Risk factors
Risk factors for BS include a genetic predisposition, with HLA-B*51 identified as a significant genetic risk factor. Genome-wide association studies have also revealed associations with the IL23R-IL12RB2 and IL10 loci. Despite these genetic associations, BS can manifest in individuals lacking these genetic markers, suggesting the potential influence of environmental factors in disease development.
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Disease course
Clinically, BS is characterized by recurrent oral ulcers, ocular inflammation, and skin lesions. Other systems, such as the gastrointestinal and musculoskeletal systems, can also be involved. Neurological involvement, known as neuro-Behcet's syndrome, is a serious manifestation of the disease and can lead to significant morbidity.
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Prognosis and risk of recurrence
The prognosis of BS is variable and unpredictable. It typically follows a relapsing and remitting course, with periods of exacerbation and remission. Over time, progressive organ damage can occur, particularly in cases with neurological or vascular involvement.
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Guidelines

Key sources

The following summarized guidelines for the evaluation and management of Behçet's syndrome are prepared by our editorial team based on guidelines from the British Association of Dermatologists (BAD/BSR 2024), the American College of Rheumatology (ACR 2023), and the European League Against Rheumatism (EULAR 2023,2018).
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Screening and diagnosis

Diagnostic criteria: as per BAD/BSR 2024 guidelines, consider using the criteria of the 1990 International Study Group or the International Criteria for Behcet's Disease for diagnosing BS.
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International Study Group (ISG) diagnostic criteria for Behçet's syndrome
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Major criteria
Recurrent oral ulcers major or minor aphthous ulcers or herpetiform ulcers) occurring ≥ 3 times per 1 year
Minor criteria
Recurrent genital ulcers aphthous or scarring)
Eye lesions anterior or posterior uveitis, cells in the vitreous on slit lamp exam, or retinal vasculitis)
Skin lesions erythema nodosum, pseudofolliculitis, papulopustular lesions, or acneiform nodules in post-adolescent patients not on steroids)
Positive pathergy read at 24-28 hours
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Classification and risk stratification

Assessment of disease activity: as per BAD/BSR 2024 guidelines, use the Physician's Global Assessment and patient-reported outcomes or a Behçet's-specific disease activity tool when assessing overall disease activity.
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Diagnostic investigations

Pretreatment evaluation: as per EULAR 2023 guidelines, screen for HBV infection in all patients eligible for treatment with conventional synthetic, biological, or targeted synthetic DMARDs, immunosuppressants, or corticosteroids (according to dose and duration).
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  • Assessment of QoL

Medical management

General principles: as per BAD/BSR 2024 guidelines, use a multidisciplinary team approach involving core specialties and any other relevant specialties (dermatology, oral medicine, ophthalmology, gastroenterology, rheumatology, and clinical psychology, with neurology and vascular surgery input where appropriate) when managing patients with BS. Manage pediatric and young patients with BS within a multidisciplinary team appropriate to their age and development. Consider referring patients to a national Behçet's center to optimize diagnosis and treatment.
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  • Management of mucocutaneous lesions (topical therapy)

  • Management of mucocutaneous lesions (systemic therapy)

  • Management of uveitis

  • Management of venous thrombosis

  • Management of arterial involvement (pharmacotherapy)

  • Management of arterial involvement (surgery)

  • Management of gastrointestinal involvement (evaluation)

  • Management of gastrointestinal involvement (corticosteroids)

  • Management of gastrointestinal involvement (immunosuppressants)

  • Management of gastrointestinal involvement (resistant disease)

  • Management of gastrointestinal involvement (surgery)

  • Management of CNS involvement (evaluation)

  • Management of CNS involvement (pharmacotherapy)

  • Management of arthritis (colchicine)

  • Management of arthritis (corticosteroids)

  • Management of arthritis (NSAIDs)

  • Management of arthritis (immunosuppressants)

  • Management of arthritis (specialist referral)

Nonpharmacologic interventions

Psychological support: as per BAD/BSR 2024 guidelines, include a clinical psychologist in the multidisciplinary team. Screen and monitor patients with BS routinely by all relevant healthcare professionals.
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Specific circumstances

Pregnant patients: as per BAD/BSR 2024 guidelines, manage pregnant patients with BS in a multidisciplinary team setting, with both a physician and obstetrician with interests in rheumatology and perinatal medicine.
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Patient education

General counseling: as per BAD/BSR 2024 guidelines, provide a patient information leaflet at the time of diagnosis and engage in conversation about BS throughout the treatment pathway to ensure shared decision-making.
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Preventative measures

Routine immunizations: as per ACR 2023 guidelines, consider offering high-dose or adjuvanted influenza vaccination, rather than regular-dose influenza vaccination, in ≥ 65 years old patients with rheumatic or musculoskeletal diseases and in 18-65 years old patients with rheumatic or musculoskeletal diseases on immunosuppressive medications.
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  • Prophylaxis for P. jirovecii pneumonia

Follow-up and surveillance

Transition to adult care: as per BAD/BSR 2024 guidelines, decide on transferring to adult care at a time of relative stability for the young patient and not based on a rigid age threshold, ensuring the transition is developmentally appropriate and follows a person-centered approach.
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  • Clinical follow-up