What is the workup for Behcet's (Behcet's disease)?

From the Guidelines

The workup for Behçet's disease involves a comprehensive clinical evaluation, including a complete history and physical examination, laboratory tests, and imaging studies, with the goal of promptly suppressing inflammatory exacerbations and preventing irreversible organ damage, as recommended by the 2018 EULAR guidelines 1. The initial assessment should include a complete history focusing on recurrent oral and genital ulcers, eye symptoms, skin lesions, joint pain, and neurological symptoms.

• Physical examination should document oral and genital ulcers, skin lesions like erythema nodosum, and eye findings.
• Laboratory tests should include complete blood count, inflammatory markers (ESR, CRP), and HLA-B51 testing, though this genetic marker is present in only 50-60% of patients.
• The pathergy test, which involves pricking the skin with a sterile needle and observing for pustule formation after 24-48 hours, can be helpful but has variable sensitivity. Ophthalmologic evaluation is essential to assess for uveitis, retinal vasculitis, or other ocular manifestations.
• Additional imaging studies like MRI for neurological involvement, colonoscopy for gastrointestinal symptoms, or vascular studies for suspected vascular involvement should be ordered based on clinical presentation. Diagnosis ultimately relies on clinical criteria, such as the International Criteria for Behçet's Disease (ICBD), which requires a score based on the presence of specific manifestations including oral aphthosis, genital aphthosis, ocular lesions, skin lesions, vascular manifestations, and positive pathergy test, as supported by the 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines 1. Treatment should be individualized according to age, gender, type and severity of organ involvement, and patients' preferences, with the goal of preventing serious complications like blindness or vascular events, as recommended by the 2018 EULAR guidelines 1. The management of Behçet's disease may involve the use of immunosuppressives, such as azathioprine and systemic corticosteroids, for patients with inflammatory eye disease affecting the posterior segment, as recommended by the 2008 EULAR recommendations 1.
• For patients with severe eye disease, the use of ciclosporine A or infliximab in combination with azathioprine and corticosteroids may be recommended.
• The management of major vessel disease in Behçet's disease is not well established, but immunosuppressive agents such as corticosteroids, azathioprine, cyclophosphamide, or ciclosporine A may be used for the management of acute deep vein thrombosis. Early diagnosis and treatment are crucial to prevent serious complications and improve quality of life for patients with Behçet's disease, as supported by the 2018 EULAR guidelines 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. The diagnosis of Behcet's disease is supported by clinical criteria and requires the exclusion of other diagnoses based on clinical presentation 2.

Clinical Evaluation

• Oral aphthous ulcers, genital ulcers, ocular lesions, and other systemic manifestations are the main clinical features of Behcet's disease 2
• A thorough medical history and physical examination are essential to identify the presence of these symptoms
• The severity of each patient's condition can be associated with a severity score according to the entire spectrum of disease manifestations 3

Laboratory Tests

• There are no pathognomonic laboratory findings for Behcet's disease 2
• Laboratory tests such as complete blood count, erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP) can be used to assess disease activity and monitor response to treatment 3, 4
• Mean corpuscular hemoglobin (MCH) and mean corpuscular hemoglobin concentration (MCHC) have been identified as valuable parameters for assessing disease severity and vascular involvement in Behcet's disease 3
• Platelet count and mean platelet volume (MPV) can also be affected in patients with Behcet's disease, and colchicine treatment has been shown to decrease MPV levels 5

Imaging Studies

• Imaging studies such as ultrasound, computed tomography, and magnetic resonance imaging can be used to evaluate vascular involvement and other systemic manifestations of Behcet's disease
• Volcano-shaped ulcers in the ileocecum are a typical finding of intestinal Behcet's disease, and punched-out ulcers can be observed in the intestine or esophagus 6

Diagnostic Criteria

• The Japanese diagnostic criteria for Behcet's disease classify intestinal Behcet's disease as a specific disease type 6
• The diagnosis of Behcet's disease requires the exclusion of other diagnoses based on clinical presentation, and there are no specific laboratory diagnostic markers for the disease 2