Beh & ccedil;et's syndrome is a rare, chronic multisystemic inflammatory disorder also known as the Silk Route disease due to its geographical distribution. Beh & ccedil;et's syndrome is a multifactorial disease and infectious, genetic, epigenetic, and immunological factors contribute to its pathogenesis. Its heterogeneous spectrum of clinical features include mucocutaneous, articular, ocular, vascular, neurological, and gastrointestinal manifestations that can present with a relapsing and remitting course. Differential diagnosis is often hampered by the non-specific clinical presentation and the absence of laboratory biomarkers or pathognomonic histological features. The therapeutic approach is tailored on the basis of patient-specific manifestations and relies on glucocorticoids, colchicine, and traditional and biological immunosuppressants. Despite progress in the knowledge and management of the disease, unmet needs in diagnostics, monitoring, prediction, and treatment personalisation challenge clinical practice, making Beh & ccedil;et's syndrome a complex disorder associated with an increased risk of morbidity.

Behçet's syndrome / Emmi, Giacomo; Bettiol, Alessandra; Hatemi, Gülen; Prisco, Domenico. - In: THE LANCET. - ISSN 0140-6736. - ELETTRONICO. - 403:(2024), pp. 1093-1108. [10.1016/s0140-6736(23)02629-6]

Behçet's syndrome

Emmi, Giacomo;Bettiol, Alessandra;Prisco, Domenico
2024

Abstract

Beh & ccedil;et's syndrome is a rare, chronic multisystemic inflammatory disorder also known as the Silk Route disease due to its geographical distribution. Beh & ccedil;et's syndrome is a multifactorial disease and infectious, genetic, epigenetic, and immunological factors contribute to its pathogenesis. Its heterogeneous spectrum of clinical features include mucocutaneous, articular, ocular, vascular, neurological, and gastrointestinal manifestations that can present with a relapsing and remitting course. Differential diagnosis is often hampered by the non-specific clinical presentation and the absence of laboratory biomarkers or pathognomonic histological features. The therapeutic approach is tailored on the basis of patient-specific manifestations and relies on glucocorticoids, colchicine, and traditional and biological immunosuppressants. Despite progress in the knowledge and management of the disease, unmet needs in diagnostics, monitoring, prediction, and treatment personalisation challenge clinical practice, making Beh & ccedil;et's syndrome a complex disorder associated with an increased risk of morbidity.
2024
403
1093
1108
Goal 3: Good health and well-being
Emmi, Giacomo; Bettiol, Alessandra; Hatemi, Gülen; Prisco, Domenico
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Utilizza questo identificatore per citare o creare un link a questa risorsa: https://hdl.handle.net/2158/1381495
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