Basaran Yilmaz syndrome
Overview
Behçet disease is a chronic condition due to disturbances in the body’s immune system. This system, which normally protects the body against infections through controlled inflammation, becomes overactive and produces unpredictable outbreaks of exaggerated inflammation.
Causes
* Argininosuccinase lyase deficiency * Argininosuccinic aciduria * Autoimmune thyroid diseases * Basaran-Yilmaz syndrome * BIDS syndrome
Diagnosis
There is no specific pathological test for Behçet disease at present. It is diagnosed clinically by specific patterns of symptoms and repeated outbreaks. Other causes for these symptoms have to be ruled out before making the diagnosis. The symptoms do not have to occur together, but can have happened at any time.
Treatment
Current treatment is aimed at easing the symptoms, reducing inflammation, and controlling the immune system. Anti-TNF therapy such as infliximab has shown promise in treating the uveitis associated with the disease. Another Anti-TNF agent, Etanercept, may be useful in patients with mainly skin and mucosal symptoms