What is Behçet's disease?

From the Guidelines

Behçet's disease is a chronic, relapsing, and remitting condition characterized by a constellation of symptoms, including mucocutaneous, joint, eye, vascular, neurological, and gastrointestinal involvement.

Key Characteristics

• The disease typically runs a relapsing and remitting course, with the goal of treatment being to promptly suppress inflammatory exacerbations and recurrences to prevent irreversible organ damage 1.
• A multidisciplinary approach is necessary for optimal care, with treatment individualized according to age, gender, type, and severity of organ involvement, as well as patient preferences 1.
• Ocular, vascular, neurological, and gastrointestinal involvement may be associated with a poor prognosis, while disease manifestations may ameliorate over time in many patients 1.

Disease Manifestations

• Mucocutaneous and joint involvement can cause impairment of quality of life but do not typically cause permanent damage, whereas untreated eye, vascular, nervous system, and gastrointestinal system involvement can cause serious damage and even death 1.
• Chronic oral and genital ulceration can cause scarring, and vigorous treatment is required to prevent oropharyngeal narrowing and obliterative and deforming genital scarring 1.

Treatment Approach

• Immunosuppressives are usually necessary to rapidly suppress inflammation and prevent relapses in patients with organ involvement 1.
• Treatment may be tapered and even stopped during the course of the disease as disease manifestations usually abate over time 1.

From the Research

Definition and Characteristics of Behçet's Disease

• Behçet's disease (BD) is a chronic, relapsing, inflammatory multisystem disease of unknown etiology 2, 3, 4, 5, 6.
• The disease has a wide clinical spectrum of mucocutaneous lesions and ocular, vascular, articular, neurologic, gastrointestinal, and cardiac involvement 2, 3, 4, 5, 6.
• BD is characterized by recurrent mucocutaneous lesions and major organ disease, such as ocular, neurologic, vascular, and gastrointestinal manifestations 3, 4, 5, 6.

Clinical Features and Diagnosis

• The clinical spectrum includes oral and genital ulcerations, uveitis, vascular, neurological, articular, renal, and gastrointestinal manifestations 4, 5.
• Diagnosis is mainly based on clinical manifestations after ruling out other potential causes, with no specific laboratory, histopathologic, or genetic findings for the diagnosis of BD 3, 4, 5.
• The International Study Group (ISG) criteria set is still the most widely used set for the diagnosis, but it has limitations, such as the lack of major organ manifestations 3.

Treatment and Management

• Treatment is mainly based on the suppression of inflammatory attacks of the disease using immunomodulatory and immunosuppressive agents 2, 5, 6.
• The primary goal of treatment should be the prevention of irreversible organ damage, with early diagnosis and appropriate treatment and close follow-up being mandatory to reduce morbidity and mortality 2.
• Treatment varies depending on the organ involved and the severity of the involvement, requiring a personalized and multidisciplinary approach 2.