PW02-004 - Autoinflammatory syndromes: a clinical review

Excerpt

Autoinflammatory syndromes are a group of rare conditions that cause intermittent episodes of fever and organ system inflammation. The majority of these conditions have been linked to single gene mutations that are involved in the acute inflammatory response. Many of these monogenic disorders, such as Familial Mediterranean Fever (FMF) and Hyper IgD syndrome (HIDS), are more prevalent in certain geographic locations such as Europe and the Mediterranean basin. It is therefore not surprising that the literature describing these conditions has largely originated from these regions. Research on these conditions so far suggests variable presentations due to a variety of modifiers such as genetic polymorphisms and/or environment. There is currently a paucity of studies from mixed populations, such as those found in urban North American centers. …