Clinical Manifestations and Diagnosis of Behçet’s Syndrome

• Main Authors: Sara Beça, Gerard Espinosa
• Format: Article
• Language: English
• Published: European Medical Journal 2021-07-01
• Series: European Medical Journal Rheumatology
• Subjects: behçet's syndrome; clinical manifestations; diagnosis; hla-b51; pathergy test
• Online Access: https://www.emjreviews.com/rheumatology/article/clinical-manifestations-and-diagnosis-of-behcets-syndrome-j170121/

Behçet’s syndrome (BS) is a systemic vasculitis with a wide range of clinical presentations and disease courses. It may involve the mucosa, skin, joints, vessels, eyes, and nervous and gastrointestinal systems. These organ involvements may present alone or co-exist in the same patient. Three main clusters of commonly co-existing manifestations were recognised and are currently called disease phenotypes. There is a significant heterogeneity among patients regarding demographic features and clinical expression of the disease that hinders a standardised disease assessment and a generalised use of diagnostic criteria. Additionally, BS is not associated with pathognomonic laboratory or histopathology features; therefore, the diagnosis is mainly based on the clinical manifestations. The purpose of this narrative review of the literature is to provide a description of the most common or typical clinical features of BS, summarise the major phenotypes of BS, and address the diagnosis strategy of this syndrome.