From the Guidelines
The workup for Behcet's disease involves a comprehensive clinical evaluation, including a detailed medical history and physical examination, laboratory tests, and specialized testing, with the goal of promptly suppressing inflammatory exacerbations and recurrences to prevent irreversible organ damage, as recommended by the 2018 update of the EULAR recommendations for the management of Behçet's syndrome 1. The process typically begins with a detailed medical history and physical examination, focusing on the characteristic recurrent oral ulcers (at least three episodes in 12 months) along with other manifestations such as genital ulcers, eye lesions, skin lesions, or vascular involvement. Some key points to consider in the workup include:
- Laboratory tests, such as complete blood count, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and basic metabolic panel, to assess for systemic inflammation.
- Specialized testing, including pathergy testing, where a sterile needle prick is observed for an exaggerated inflammatory response after 24-48 hours.
- Ophthalmologic examination to evaluate for uveitis, retinal vasculitis, or other ocular manifestations.
- Additional imaging studies, such as MRI for neurological involvement, vascular studies for large vessel disease, or colonoscopy for intestinal involvement, may be necessary depending on symptoms.
- HLA-B51 genetic testing may support the diagnosis but is not diagnostic alone. The diagnosis ultimately relies on clinical criteria, such as the International Criteria for Behcet's Disease (ICBD), which uses a point-based system for various manifestations, with a score of 4 or more supporting the diagnosis, as noted in the 2018 update of the EULAR recommendations 1. Early diagnosis is important as prompt treatment can prevent serious complications like blindness or vascular damage, and a multidisciplinary approach is necessary for optimal care, as emphasized in the 2018 update of the EULAR recommendations 1. It is also important to consider the individualized treatment approach, taking into account the patient's age, gender, type and severity of organ involvement, and patient's preferences, as recommended by the 2018 update of the EULAR recommendations 1.
From the Research
Workup for Behcet's Disease
The workup for Behcet's disease involves a combination of clinical evaluation, laboratory tests, and imaging studies to confirm the diagnosis and assess the extent of organ involvement. The key features of the workup include:
- Clinical evaluation: A thorough medical history and physical examination to identify the characteristic symptoms of Behcet's disease, such as oral and genital ulcers, skin lesions, and eye inflammation 2, 3, 4.
- Laboratory tests: Although there is no specific laboratory test for Behcet's disease, tests such as complete blood count, erythrocyte sedimentation rate, and C-reactive protein may be used to assess inflammation and rule out other conditions 2, 5.
- Imaging studies: Imaging tests such as computed tomography (CT) scans and magnetic resonance imaging (MRI) may be used to evaluate the extent of organ involvement, particularly in the gastrointestinal and central nervous systems 6, 5.
- Pathergy test: A positive pathergy test, which involves pricking the skin with a needle to assess the inflammatory response, may be used to support the diagnosis of Behcet's disease 6, 5.
- HLA-B51 testing: Testing for HLA-B51, a genetic marker associated with Behcet's disease, may be used to support the diagnosis, particularly in patients with a strong clinical suspicion of the disease 2, 5.
Diagnostic Criteria
The diagnosis of Behcet's disease is based on clinical criteria, as there is no single laboratory test or imaging study that can confirm the diagnosis. The International Study Group diagnostic criteria, published in 1990, are widely used to diagnose Behcet's disease 4, 5. The criteria require the presence of recurrent oral ulcers and at least two of the following: genital ulcers, typical eye lesions, typical skin lesions, and a positive pathergy test.
Differential Diagnosis
The differential diagnosis for Behcet's disease includes other conditions that can cause similar symptoms, such as:
- Sweet's disease
- Pemphigus
- Erythema nodosum
- Crohn's disease 5 A thorough clinical evaluation and laboratory tests are necessary to rule out these conditions and confirm the diagnosis of Behcet's disease.