Behçet syndrome: from pathogenesis to novel therapies

Abstract

Behçet syndrome is a chronic disease hallmarked by inflammation of the blood vessels that is related to an autoimmune reaction caused by inherited susceptibility due to specific genes and environmental factors, probably components of infectious microorganisms, which turn on or get going the disease in genetically susceptible subjects. The more common clinical expression of the disease is represented by a triple-symptom complex of recurrent oral aphthous ulcers, genital ulcers, and uveitis, sometimes associated with inflammatory arthritis, phlebitis, iritis, as well as inflammation of the digestive tract, brain, and spinal cord. The treatment strategies used to manage the manifestations of Behçet syndrome have gradually progressed, and a number of new therapeutic resources have been implemented in recent years, allowing better control of pathogenic mechanisms, reducing symptoms and suffering, and ameliorating patient's outcome.

Donor IFNL4 Genotype Is Associated with Early Post-Transplant Fibrosis in Recipients with Hepatitis C

“…In addition, polymorphisms in both IFNL3 and IFNL4 have been shown to be associated with high serum and hepatic iron concentrations [10]. Though there is only a weak association between response to direct active anti-viral therapy (DAA) and IFNL4, the presence of the beneficial IFNL4 polymorphisms were associated with higher prevalence of the NS5A Y93H resistance associated variant (RAV) [11,12].…”

Intestinal Behçet and Crohn’s disease: two sides of the same coin

“…The annual incidence of CD varies from 0 to 20.2 per 100,000 in North America and 0.3 – 12.7 per 100,000 in Europe [11]. It is highest in Western countries in young individuals aged 15 to 29 years [7, 12]. …”

“…The manifestations of BD are similar to those of IBD, specifically uveitis, arthritis, oral ulcers, pyoderma gangrenosum, vaso-occlusive disease, and thrombotic events [12]. Uveitis in BD can be characterized by chronic pan-uveitis or posterior uveitis with necrotizing retinal vasculitis and tends to be more recurrent and sight threatening than other endogenous uveitis.…”

Vascular Manifestations of Behçet’s Disease

“…Therefore, many factors play a role in the pathogenesis of BD. Vascular damage triggered by inflammation increases the risk of thrombosis [41]. Increased plasma levels of nitric oxide (NO) and its metabolites seen in BD patients demonstrate also the presence of an endothelial dysfunction [54].…”

“…Changes in endothelial function due to this involvement cause different grades of clinically observable organ lesions [41]. Vascular involvement, especially arterial involvement, is one of the major causes of morbidity and mortality [42].…”