Vasculopathy, Behçet's syndrome, and familial Mediterranean fever.

Within the past year information has accumulated on the role of infections in the pathogenesis of vasculitis. Superantigens play an important role in the stimulation of the immune system. Behçet's syndrome is a vasculitis that affects children as well as adults. Recently published childhood studies highlight the importance of family history and geographic differences in this disease. Familial Mediterranean fever is a genetic disease manifesting frequently in children. Studies on the function of the mutated protein pyrin in this disease are in progress. Some researchers have proposed that pyrin is an anti-inflammatory protein. Understanding its mode of action will help us understand more of the general inflammatory pathway.