What is Behcet's Syndrome?

From the Guidelines

Behcet's Syndrome is a rare, chronic inflammatory disorder characterized by recurrent oral and genital ulcers, eye inflammation, and skin lesions, and its management should be individualized according to age, gender, type and severity of organ involvement, and patient preferences, as recommended by the 2018 EULAR guidelines 1.

Overview of Behcet's Syndrome

Behcet's Syndrome is a multisystem disease that can affect blood vessels, joints, the digestive tract, and the central nervous system. The disease is believed to be an autoimmune condition triggered by environmental factors in genetically susceptible individuals, particularly those with HLA-B51 gene. The diagnostic criteria for Behcet's Syndrome require oral ulceration and at least two of the following lesions: recurrent genital ulceration, uveitis or retinal vasculitis, or skin lesions, such as erythema nodosum, pseudofolliculitis, or pathergy 1.

Treatment of Behcet's Syndrome

Treatment of Behcet's Syndrome typically involves medications to reduce inflammation and suppress the immune system, such as:

• Corticosteroids
• Colchicine
• Immunosuppressants like azathioprine or cyclosporine
• Biologic agents like TNF inhibitors (infliximab, adalimumab) for severe cases The specific treatment regimen depends on which organs are affected and the severity of symptoms. For example:
• Oral ulcers can be treated with topical steroids like triamcinolone dental paste or dexamethasone elixir
• Eye involvement often requires ophthalmologist management with topical and systemic treatments
• Vascular involvement may require immunosuppressives, such as azathioprine or cyclophosphamide, and/or anti-TNF agents 1

Management Principles

The 2018 EULAR guidelines recommend the following overarching principles for the management of Behcet's Syndrome:

• The goal of treatment is to promptly suppress inflammatory exacerbations and recurrences to prevent irreversible organ damage
• A multidisciplinary approach is necessary for optimal care
• Treatment should be individualized according to age, gender, type and severity of organ involvement, and patient preferences
• Ocular, vascular, neurological, and gastrointestinal involvement may be associated with a poor prognosis, and require prompt and aggressive treatment 1

From the Research

Definition and Characteristics of Behcet's Syndrome

• Behcet's Syndrome (BS) is a chronic relapsing multisystemic inflammatory disorder characterized by four major symptoms: oral aphthous ulcers, genital ulcers, skin lesions, and ocular lesions 2.
• It is a form of vasculitis that can affect blood vessels of all sizes, with a higher prevalence in regions along the Silk Road, from the Mediterranean to the Far East 3.
• The disease can also manifest with minor symptoms, including arthritis, gastrointestinal ulcers, epididymitis, vascular lesions, and central nervous system symptoms 2.

Clinical Manifestations and Diagnosis

• The clinical manifestations of BS are protean in nature, with most patients developing mucocutaneous and genital ulcers, along with eye disease 3.
• Other patients may present with arthritis, frank vasculitis, thrombophlebitis, and CNS disease, with manifestations varying considerably based on gender and ethnicity 3.
• Diagnosis is made based on clinical manifestations, as there is no laboratory examination with diagnostic value for BS 4.
• The International Study Group diagnostic criteria published in 1990 is the most widely used and recognized, with the International Criteria for Behcet's Disease developed in 2014 to improve sensitivity 4.

Treatment and Management

• The goal of BS treatment is to eradicate triggers and/or aggravating factors, alleviate and control clinical symptoms, prevent and treat any damage to organs, slow disease progression, and improve the patient's quality of life 4.
• Treatment depends on the affected organs and disease severity, with pharmacological agents including corticosteroids, colchicine, azathioprine, and tumour necrosis factor (TNF)α inhibitors 3.
• Management of the patient with BS is complex and often requires a multidisciplinary approach, with strategies to assess and stratify patients based on clinical manifestations and disease severity 3.