From the Guidelines
Behcet's disease is a rare, chronic inflammatory disorder characterized by blood vessel inflammation, typically manifesting as recurrent mouth sores, genital ulcers, eye inflammation, and skin lesions, with a relapsing-remitting course that requires individualized treatment to prevent irreversible organ damage. The disease can affect various parts of the body, including joints, the digestive tract, nervous system, and blood vessels, and is considered an autoimmune condition with unknown exact cause, but genetic factors like the HLA-B51 gene and environmental triggers may play roles in its development 1.
Key Characteristics
- Recurrent mouth sores (aphthous ulcers)
- Genital ulcers
- Eye inflammation (uveitis)
- Skin lesions
- Joint, digestive tract, nervous system, and blood vessel involvement
Treatment Approach
Treatment depends on symptom severity and typically includes:
- Corticosteroids like prednisone
- Immunosuppressants such as azathioprine, cyclosporine, or methotrexate
- Biologic agents like infliximab or adalimumab for more severe cases
- Colchicine for mucocutaneous symptoms
- Topical steroids for oral and genital ulcers
According to the 2018 update of the EULAR recommendations for the management of Behçet's syndrome, a multidisciplinary approach is necessary for optimal care, and treatment should be individualized according to age, gender, type and severity of organ involvement, and patients' preferences 1. The goal of treatment is to promptly suppress inflammatory exacerbations and recurrences to prevent irreversible organ damage, particularly in cases with ocular, vascular, neurological, or gastrointestinal involvement, which may be associated with a poor prognosis.
From the Research
Definition and Characteristics of Behcet's Disease
- Behcet's disease (BD) is a rare systemic vasculitis characterized by oral aphthous ulcers, genital ulcers, ocular lesions, and other systemic manifestations 2.
- It occurs most frequently in Eurasian populations along the ancient trading route known as the "Silk Road" which extends from eastern Asia to the Mediterranean basin 2.
- The causes of BD are unknown, but it is believed to be due to an autoimmune process triggered by an infectious or environmental agent in genetically predisposed individuals 2.
Pathophysiology and Histology
- The HLA-B51 allele located in the MHC locus, on chromosome 6p, has been the most strongly associated risk factor for BD in areas along the Old Silk Route 2.
- Herpes simplex virus-1 and Streptococcus have been postulated as possible environmental triggers of BD 2.
- T cell homeostasis perturbation, especially Th1 and Th17 expansion and decreased regulation by Tregs, are now supposed to be the cornerstone of BD pathogenesis 2.
- The histology shows vasculitis that involves both arteries and veins, and vessels of any size, with significant neutrophil infiltration, endothelial cell swelling, and fibrinoid necrosis 2.
Clinical Features and Diagnosis
- BD is a systemic vasculitis with a wide clinical spectrum, including recurrent oral and genital ulcerations, uveitis, and vascular, neurological, articular, renal, and gastrointestinal manifestations 3.
- The diagnosis of BD is only supported by clinical criteria and requires the exclusion of other diagnoses based on clinical presentation 2.
- There are no pathognomonic laboratorial findings of BD 2.
Treatment Options
- Corticosteroids are commonly used to treat clinical manifestations of BD in combination with immunosuppressant drugs 2.
- Tumor necrosis factor (TNF)-blocking agents, such as Infliximab, Etanercept, and Adalimumab, have been reported to have success in patients with BD 2, 4.
- Other treatment options include colchicine, nonsteroidal anti-inflammatory agents, and topical treatments with corticosteroids for mucocutaneous and joint involvement 3.
- Novel targeted immunosuppressive therapies, such as interleukin (IL)-1 inhibitors, tocilizumab, rituximab, alemtuzumab, ustekinumab, interferon-alpha-2a, and apremilast, have shown promising results in the treatment of BD 5, 6.