What is Behcet's disease (Behcet's syndrome)?

From the Guidelines

Behcet's disease management should prioritize a multidisciplinary approach, individualized treatment based on symptom severity and organ involvement, and prompt suppression of inflammatory exacerbations to prevent irreversible organ damage, as recommended by the 2018 EULAR guidelines 1.

Overview of Behcet's Disease

Behcet's disease is a rare, chronic inflammatory disorder characterized by recurrent oral and genital ulcers, eye inflammation, and skin lesions. The disease results from dysregulated immune responses with genetic predisposition (particularly HLA-B51) and environmental triggers contributing to pathogenesis.

Treatment Approach

Treatment depends on symptom severity and organ involvement. For mild oral and genital ulcers, topical corticosteroids like triamcinolone 0.1% paste or dexamethasone elixir can provide relief. Colchicine (0.6-1.2 mg daily) is often used as first-line therapy for mucocutaneous symptoms, as recommended by the EULAR guidelines 1.

• Key considerations in treatment include:
  • Individualizing treatment according to age, gender, type and severity of organ involvement, and patients’ preferences.
  • Recognizing that ocular, vascular, neurological, and gastrointestinal involvement may be associated with a poor prognosis and require aggressive treatment.
  • Using immunosuppressives to rapidly suppress inflammation and prevent relapses in patients with organ involvement.

Management of Specific Manifestations

• Mucocutaneous involvement: Colchicine should be the initial treatment, with azathioprine, interferon-alpha, or TNF-alpha inhibitors considered in recurrent and chronic cases 1.
• Ocular involvement: Aggressive treatment with corticosteroids and immunosuppressants is necessary to prevent vision loss.
• Vascular manifestations: Anticoagulation alongside immunosuppression may be required.
• Leg ulcers: Management may involve immunosuppressives, antibiotics, debridement, or occlusive measures, and should be planned with a dermatologist and vascular surgeon experienced with such lesions.

Multidisciplinary Care

Regular monitoring for disease activity and medication side effects is essential, and a multidisciplinary approach involving rheumatology, ophthalmology, and dermatology provides optimal care for these patients 1.

From the Research

Definition and Diagnosis of Behcet's Disease

• Behcet's disease is a multisystem disease featuring mucocutaneous, ocular, intestinal, articular, vascular, urogenital, and neurologic involvement 2
• The diagnosis of Behcet's disease is based on clinical criteria, including recurrent aphthous ulcerations in the mouth, skin lesions, eye lesions, and genital ulcerations 2
• A nonspecific skin hyperreactivity called pathergy is said to be helpful in the diagnosis, and biopsy usually shows a venulitis 2

Treatment of Behcet's Disease

• The treatment of Behcet's disease is difficult to evaluate due to the many spontaneous exacerbations and remissions during the clinical course of the disease 2
• Topical corticosteroids for orogenital ulcers and ocular inflammation are helpful, and intralesional injections for affected joints and retrobulbar tissues are useful in some cases 2
• Systemic corticosteroids have appeared to be helpful for all manifestations of the disease, with a typical dosage of 60 mg of prednisone by mouth daily during acute exacerbations 2
• Immunomodulatory and immunosuppressive agents such as colchicine, azathioprine, cyclosporine-A, interferon-alpha, and cyclophosphamide are used as steroid-sparing agents and to prevent further relapses 3, 4
• Tumor necrosis factor alpha inhibitors, especially infliximab and adalimumab, are increasingly used for various refractory BS manifestations despite the lack of controlled studies 3, 4, 5
• Apremilast is now approved for the treatment of oral ulcer of Behcet syndrome in the United States, and its potential benefits in controlling nonoral ulcer features of the syndrome are awaited 5
• New treatment options such as ustekinumab, secukinumab, tocilizumab, and others have early promising data but more studies are needed to better clarify their role in Behcet management 5

Management of Neuro-Behcet's Syndrome

• Neuro-Behcet Syndrome (NBS) is a severe chronic inflammatory vascular disease involving the Central Nervous System (CNS), and it is an invalidating condition with disability and a huge impact on quality of life 6
• There is no evidence to support or refute the benefit of biologics, colchicine, corticosteroids, immunosuppressants, and interferon-alpha for the treatment of patients with NBS, and well-designed multicentre RCTs are needed to inform and guide clinical practice 6