From the Guidelines
Behçet's disease 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's preferences, as recommended by the 2018 update of the EULAR recommendations for the management of Behçet's syndrome 1.
Key Characteristics of Behçet's Disease
- Recurrent oral and genital ulcers
- Eye inflammation (uveitis or retinal vasculitis)
- Skin lesions (erythema nodosum, pseudofolliculitis, or pathergy)
- Vascular involvement (venous or arterial)
- Multisystem vasculitis affecting joints, digestive tract, nervous system, and blood vessels
Diagnosis and Treatment
- Diagnostic criteria established by the International Group for Behçet’s disease require oral ulceration and 2 of the 3 lesions: recurrent genital ulceration, uveitis or retinal vasculitis, or skin lesions 1
- Treatment typically involves immunosuppressive medications tailored to symptom severity, including colchicine, corticosteroids, azathioprine, cyclosporine, or TNF inhibitors like infliximab 1
- Topical steroids may help manage oral and genital ulcers
- Patients should be monitored regularly by specialists, particularly ophthalmologists, as eye involvement can lead to blindness if untreated
Management of Specific Organ Involvement
- Eye involvement: azathioprine, cyclosporine-A, interferon-alpha, or monoclonal anti-TNF antibodies, with systemic glucocorticoids used only in combination with azathioprine or other systemic immunosuppressives 1
- Vascular involvement: immunosuppressive agents such as corticosteroids, azathioprine, cyclophosphamide, or ciclosporine A, with anticoagulants, antiplatelet, or antifibrinolytic agents not recommended due to lack of evidence 1
Prognosis and Quality of Life
- Disease manifestations may ameliorate over time in many patients
- Ocular, vascular, neurological, and gastrointestinal involvement may be associated with a poor prognosis
- Treatment should aim to promptly suppress inflammatory exacerbations and recurrences to prevent irreversible organ damage and improve quality of life 1
From the Research
Definition and Overview of Behçet's Disease
- Behçet's disease (BD) is a chronic, relapsing, systemic vasculitis of unknown etiology, characterized by oral and genital ulcers, cutaneous vasculitic lesions, and involvement of various organs such as the eyes, joints, vessels, gastrointestinal tract, nervous system, and heart 2.
- It is a long-term, cyclical disease with symptom-free periods interrupted by exacerbations of varying intensities 3.
- The disease affects both genders and all ages, with a higher frequency in populations along the ancient "Silk Road" extending from Eastern Asia to the Mediterranean region 2, 3, 4, 5.
Clinical Features and Diagnosis
- Mucocutaneous manifestations, such as oral aphthae and genital ulcers, are the clinical hallmarks of BD 2.
- The diagnostic criteria are based on mucocutaneous manifestations due to their high sensitivity and specificity 2.
- The diagnosis relies largely on clinical criteria, and there are no pathognomonic laboratory findings 4, 5.
- Differential diagnosis is important to exclude other diseases such as Sweet's disease, pemphigus, erythema nodosum, and Crohn's disease 5.
Pathogenesis and Treatment
- Genetic factors, particularly HLA-B51 antigen, play a key role in the pathogenesis of BD 2, 4, 5.
- Streptococcus sanguinis or microbiome changes can trigger innate immune system-mediated inflammation, sustained by adaptive immune responses 2.
- Treatment aims to prevent irreversible organ damage and relieve symptoms, with corticosteroids, immunosuppressants, and anti-TNF agents being commonly used 2, 3, 6, 4, 5.
- Newer agents, such as apremilast and anti-TNF-α agents, have shown promise in managing BD and improving patients' quality of life and prognosis 2, 6.