Behçet's disease physiopathology: a contemporary review

• Published in: Autoimmunity highlights Vol. 7; no. 1; p. 4
• Main Authors: Zeidan, Mohamad J, Saadoun, David, Garrido, Marlene, Klatzmann, David, Six, Adrien, Cacoub, Patrice
• Format: Journal Article
• Language: English
• Published: England Springer Nature B.V 01-12-2016; Springer Verlag; Springer International Publishing
• Subjects: Central nervous system; Clinical aspects; Etiology; Gastrointestinal tract; Human health and pathology; Life Sciences; Morbidity; Review; Skin diseases; Skin lesions; Systemic vasculitis; Ulcers; Autoimmunity; Inflammation; Behçet disease; Physiopathology
• ISSN: 2038-0305; 2038-3274
• DOI: 10.1007/s13317-016-0074-1

Behçet's disease, also known as the Silk Road Disease, is a rare systemic vasculitis disorder of unknown etiology. Recurrent attacks of acute inflammation characterize Behçet's disease. Frequent oral aphthous ulcers, genital ulcers, skin lesions and ocular lesions are the most common manifestations. Inflammation is typically self-limiting in time and relapsing episodes of clinical manifestations represent a hallmark of Behçet's disease. Other less frequent yet severe manifestations that have a major prognostic impact involve the eyes, the central nervous system, the main large vessels and the gastrointestinal tract. Behçet's disease has a heterogeneous onset and is associated with significant morbidity and premature mortality. This study presents a current immunological review of the disease and provides a synopsis of clinical aspects and treatment options.