What is Behcet's disease?

From the Guidelines

Treatment of Behcet's disease should be individualized according to age, gender, type and severity of organ involvement, and patients' preferences, with the goal of promptly suppressing inflammatory exacerbations and recurrences to prevent irreversible organ damage. Behcet's disease is a condition that typically runs a relapsing and remitting course, and a multidisciplinary approach is necessary for optimal care 1. The disease manifestations may ameliorate over time in many patients, but ocular, vascular, neurological, and gastrointestinal involvement may be associated with a poor prognosis.

When there is only skin, mucosa, and joint involvement, treatment can be tailored according to the patient's need and how much the symptoms impact on their quality of life compared with the risks associated with adverse effects of any medication used 1. However, when chronic oral and genital ulceration causes scarring, vigorous treatment is required to prevent oropharyngeal narrowing and obliterative and deforming genital scarring.

Key considerations in managing Behcet's disease include:

• Individualized treatment based on the severity and type of organ involvement
• Prompt suppression of inflammatory exacerbations to prevent irreversible organ damage
• Regular monitoring for disease activity and medication side effects
• A multidisciplinary approach to care, particularly for patients with ocular, vascular, neurological, or gastrointestinal involvement
• Consideration of the patient's quality of life and preferences in treatment decisions 1.

In patients with severe disease, particularly those with organ involvement, immunosuppressives are usually necessary to rapidly suppress inflammation and prevent relapses, with the goal of preventing loss of function 1. The treatment may be tapered and even stopped during the course of the disease as the disease manifestations usually abate over time.

From the Research

Overview of Behcet's Disease

• Behcet's disease (BD) is a chronic, relapsing inflammatory, multisystem disease of unknown etiology 2
• The disease has a wide clinical spectrum of mucocutaneous lesions and ocular, vascular, articular, neurologic, gastrointestinal, and cardiac involvement 2

Treatment of Behcet's Disease

• The primary goal of treatment should be the prevention of irreversible organ damage 2
• Treatment varies depending on the organ involved and the severity of the involvement 2
• Treatment is mainly based on suppression of the inflammatory attacks of the disease using local and systemic immunomodulatory and immunosuppressive drugs 2
• Corticosteroids are the cornerstone of therapy for inducing remission, whereas immunomodulatory and immunosuppressive agents are used as steroid-sparing agents and to prevent further relapses 3

Neurologic Complications of Behcet's Disease

• Clinical and imaging data suggest that Behcet's disease can present with a variety of neurologic complications, which may be subclassified into two forms: Central Nervous System-Neuro-Behçet syndrome (CNS-NBS) and cerebral venous sinus thrombosis 4
• Patients with small vein inflammation should be considered for aggressive treatment, and acute attacks of CNS-NBS are treated with either oral prednisone or high-dose intravenous methylprednisolone (IVMP) 4

Ocular Manifestations of Behcet's Disease

• Ocular lesions of Behcet's disease need aggressive treatment to prevent severe loss of vision or blindness 5
• A combination of pulse cyclophosphamide and azathioprine has been shown to be effective in treating ocular manifestations of Behcet's disease, with significant improvements in visual acuity, posterior uveitis, and retinal vasculitis 5

Use of Anti-TNF Alpha Agents in Behcet's Disease

• Anti-TNF alpha agents, such as infliximab and adalimumab, are increasingly used for various refractory BS manifestations, despite the lack of controlled studies 3
• A multicenter study of 124 patients with severe and/or refractory manifestations of Behcet's disease found that anti-TNF alpha therapy is efficient in all severe and refractory BD manifestations, with an overall response rate of 90.4% 6