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Behçet's syndrome

Background

Overview

Definition
Behcet's syndrome 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 Behcet's syndrome 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 Behcet's syndrome in the United Kingdom is estimated at 0.64 per 100,000 population.
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Disease course
Clinically, Behcet's syndrome 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 Behcet's syndrome 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 American College of Rheumatology (ACR 2023) and the European League Against Rheumatism (EULAR 2022,2018)....
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Diagnostic investigations

Pretreatment evaluation: as per EULAR 2022 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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Medical management

Management of mucocutaneous lesions: as per EULAR 2018 guidelines, Initiate topical corticosteroids for the treatment of oral and genital ulcers.
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  • Management of uveitis

  • Management of venous thrombosis

  • Management of arterial aneurysms

  • Management of gastrointestinal involvement

  • Management of CNS involvement

  • Management of arthritis

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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