From the Guidelines
The workup for Behçet's disease involves a multidisciplinary approach with a comprehensive evaluation, including a thorough medical history, physical examination, and laboratory tests to promptly suppress inflammatory exacerbations and recurrences and prevent irreversible organ damage 1.
Key Components of Workup
- A thorough medical history to identify the type and severity of organ involvement
- Physical examination to assess the extent of disease manifestations
- Laboratory tests, such as complete blood count, erythrocyte sedimentation rate, and C-reactive protein, to evaluate disease activity
- Imaging studies, including ocular and vascular assessments, to evaluate disease extent and severity 1
Treatment Approach
Treatment should be individualised according to age, gender, type and severity of organ involvement and patients’ preferences 1.
- For patients with 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
- For patients with organ involvement, such as ocular, vascular, neurological, or gastrointestinal involvement, immunosuppressives are usually necessary to rapidly suppress the inflammation and prevent relapses in order to prevent loss of function 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
Diagnosis of Behçet's Disease
The diagnosis of Behçet's disease (BD) is largely based on the International Study Group (ISG) criteria, which are more specific than sensitive 2. However, these criteria do not include intestinal manifestations, a feature more commonly seen in Western patients.
Clinical Manifestations
The clinical manifestations of BD can vary among geographic and ethnic populations 2. Common manifestations include:
- Recurrent oral and genital ulcers
- Skin lesions
- Ocular involvement
- Gastrointestinal involvement
- Vascular involvement
- Neurological involvement
- Joint involvement
Laboratory Tests
Laboratory tests that may be used to support the diagnosis of BD include:
- Elevated inflammatory markers such as C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) 2, 3, 4
- Complete blood count (CBC) to evaluate for anemia, leukocytosis, or thrombocytosis
- Blood chemistry tests to evaluate for liver or kidney dysfunction
- Immunological tests such as antinuclear antibody (ANA), rheumatoid factor (RF), and anti-neutrophil cytoplasmic antibody (ANCA) to rule out other autoimmune disorders
Imaging Studies
Imaging studies that may be used to support the diagnosis of BD include:
- Magnetic resonance imaging (MRI) or computed tomography (CT) scans to evaluate for central nervous system involvement 5
- Magnetic resonance venography or computerized tomographic angiography to diagnose extraparenchymal neuro-Behçet's disease 5
Histopathological Analysis
Histopathological analysis of tissue biopsies may be used to support the diagnosis of BD, particularly in cases with intestinal involvement 3.
Risk Factors for Disease Activity
Risk factors for disease activity in BD patients include:
- Disease duration ≤ 60 months
- Neutrophil-to-lymphocyte ratio (NLR) ≥ 2
- CRP ≥ 10 mg/L
- ESR ≥ 20 mm/H
- Albumin-to-globulin ratio (AGR) < 1.5 4