From the Guidelines
Diagnosing Behcet's disease requires a comprehensive clinical evaluation based on the International Criteria for Behcet's Disease (ICBD) scoring system, which includes oral aphthosis, genital aphthosis, ocular manifestations, skin lesions, neurological manifestations, vascular manifestations, and positive pathergy test. The diagnosis is primarily clinical, relying on the recognition of a pattern of recurrent symptoms, including recurrent oral ulcers, genital ulcers, eye lesions, and skin lesions 1. According to the ICBD scoring system, oral aphthosis, genital aphthosis, and ocular manifestations each score 2 points, while skin lesions, neurological manifestations, vascular manifestations, and positive pathergy test each score 1 point; a score of 4 or more indicates Behcet's disease.
Some key points to consider in the diagnosis of Behcet's disease include:
- Recurrent oral ulcers (at least three times in 12 months)
- Recurrent genital ulcers
- Eye lesions (uveitis, retinal vasculitis)
- Skin lesions (erythema nodosum, pseudofolliculitis, papulopustular lesions)
- Positive pathergy test Laboratory tests like complete blood count, inflammatory markers (ESR, CRP), and HLA-B51 genetic testing may support the diagnosis, though HLA-B51 positivity alone is not diagnostic 1. Specialized tests including ophthalmologic examination, skin pathergy testing, and imaging studies (MRI for neurological involvement, vascular studies) are often necessary to confirm the diagnosis and rule out other conditions.
It is essential to rule out other diseases that may mimic Behcet's disease, such as inflammatory bowel disease, reactive arthritis, and other vasculitides, before confirming the diagnosis 1. A comprehensive clinical evaluation, including a thorough medical history, physical examination, and laboratory tests, is crucial in making an accurate diagnosis of Behcet's disease. The ICBD scoring system provides a useful framework for diagnosing Behcet's disease, and a score of 4 or more indicates a high likelihood of the disease.
From the Research
Diagnostic Criteria for Behcet's Disease
- The diagnosis of Behcet's disease is mainly based on clinical manifestations after ruling out other potential causes 2
- There are no specific laboratory, histopathologic, or genetic findings for the diagnosis of Behcet's disease 2, 3
- The International Study Group (ISG) criteria set is still the most widely used set for the diagnosis, but it has limitations, such as the lack of major organ manifestations 2
- The ICBD 2014 criteria are more sensitive, especially in early disease, but may cause overdiagnosis in patients with similar conditions, such as spondyloarthritis 2
Clinical Manifestations and Diagnostic Tools
- Recurrent oral ulceration is an obligatory manifestation for the diagnosis of Behcet's disease, according to the ISG criteria 3
- The pathergy test is the only diagnostic test used as diagnostic or classification criteria for Behcet's disease 2
- Common femoral vein (CFV) thickness measurement may be a valuable diagnostic tool for Behcet's disease, with sensitivity and specificities higher than 80% for the cut-off value of 0.5 mm 2
- Volcano-shaped ulcers in the ileocecum are a typical finding of intestinal Behcet's disease 4
Laboratory Tests and Biomarkers
- There are no specific laboratory diagnostic markers for Behcet's disease, making diagnosis more difficult 5, 6
- C-reactive protein (CRP), mean corpuscular hemoglobin (MCH), mean corpuscular hemoglobin concentration (MCHC), neutrophil count, platelet count, and plateletcrit may be increased in Behcet's disease patients compared to healthy controls 6
- MCH and MCHC may be valuable parameters for assessing disease severity and indicating vascular involvement in Behcet's disease 6