From the Guidelines
The workup for Behcet's disease should involve a comprehensive clinical evaluation and laboratory testing, focusing on documenting characteristic manifestations and excluding other conditions that may mimic Behcet's disease, as recommended by the 2018 update of the EULAR recommendations for the management of Behçet's syndrome 1.
Key Components of the Workup
- A detailed history focusing on recurrent oral and genital ulcers, eye symptoms, skin lesions, joint pain, and neurological symptoms
- Physical examination to document oral aphthous ulcers, genital ulcers, skin lesions (erythema nodosum, pseudofolliculitis), and eye findings
- Laboratory tests, including:
- Complete blood count
- Inflammatory markers (ESR, CRP)
- HLA-B51 testing (positive in 50-60% of patients but not diagnostic alone) 1
- Additional testing, such as:
- Pathergy test (skin prick that produces papule/pustule in 24-48 hours)
- Ophthalmologic examination for uveitis
- Imaging studies like MRI for neurological involvement or vascular studies if vascular Behcet's is suspected
- Skin or mucosal biopsies may help exclude other conditions
Diagnosis
Diagnosis is primarily clinical, based on the International Criteria for Behcet's Disease (ICBD), requiring a score of ≥4 points from various manifestations, including:
- Oral aphthosis (2 points)
- Genital aphthosis (2 points)
- Ocular lesions (2 points)
- Skin lesions (1 point)
- Vascular manifestations (1 point)
- Positive pathergy test (1 point) A multidisciplinary approach is necessary for optimal care, and treatment should be individualised according to age, gender, type and severity of organ involvement, and patient’s preferences, as recommended by the 2018 EULAR guidelines 1.
From the Research
Behcet Work Up
- The diagnosis of Behcet's syndrome (BS) is made based on clinical manifestations, as there is no laboratory examination with diagnostic value for BS 2.
- The International Study Group diagnostic criteria published in 1990 is the most widely used and recognized, but the International Criteria for Behcet's Disease developed in 2014 can improve sensitivity 2.
- Evaluating disease activity in BS is important for treatment selection and monitoring, and the simplified Behcet's Disease Current Activity Form (2006 version) is a well-established scoring method 2.
Laboratory Tests
- Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) can be used to predict active Behcet's disease (BD) and active vascular manifestations 3.
- Patients with active oral, genital, joint, and dermal manifestations had higher ESR and CRP values 3.
- The optimal value of ESR ≥ 10.5 and ESR ≥ 42.5 could predict active BD and active vascular manifestations with sensitivity and specificity 3.
Disease Activity and Organ Involvement
- Red blood cell distribution width (RDW) may be a cost-effective, novel potential parameter to evaluate disease activity in Behcet's disease 4.
- RDW was increased in active disease compared with inactive disease, but no relationships were found between organ involvement and RDW 4.
- Intestinal Behcet's disease can be diagnosed based on typical findings such as volcano-shaped ulcers in the ileocecum, and tumor necrosis factor inhibitors can be used as standard therapy 5.
Treatment
- The goal of BS treatment is to eradicate triggers and/or aggravating factors, alleviate and control clinical symptoms, prevent and treat any damage to organs, slow disease progression, and improve the patient's quality of life 2.
- Colchicine can suppress platelet function and be used in vascular involvement together with immunosuppressant agents in Behcet's Disease 6.