An update on autoinflammatory diseases. New concepts for new and old diseases.
The discovery of MEFV as the susceptibility gene for autosomal recessive Familial Mediterranean Fever (FMF) in 1997 represents the beginning of the new era of the monogenic autoinflammatory diseases. During the last decade, the increasing knowledge on the pathogenic mechanisms related to a number of diseases associated to mutations of genes associated to autoinflammatory diseases had a terrific impact on the understanding of pivotal mechanisms regulating the inflammatory response and therefore represents one of the major advance in the field of inflammation.The International Congress on Familiar Mediterranean Fever and Systemic Autoinflammatory Diseases brings together the experts in the field every two and a half years and represents a unique opportunity for an update on the recent progress in this growing field. The fifth edition of the congress was held in Rome (Italy, 4-8 April 2008). Most of the contributions to this meeting have been published during the course of the present year. Thus, the aim of the present article is to report the main highlights from the above-mentioned meeting and to give a general update of the more recent advances in this field.
Gattorno
M
M
U.O. Pediatria 2, IRCCS G. Gaslini and Department of Pediatrics, University of Genoa, Italy. marcogattorno@ospedale-gaslini.ge.it
La Regina
M
M
Martini
A
A
Manna
R
R
D048868:Adaptor Proteins, Signal Transducing / Q000235:genetics; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D051017:Apoptosis Regulatory Proteins / Q000235:genetics; D001327:Autoimmune Diseases / Q000188:drug therapy / Q000235:genetics* / Q000276:immunology; D003598:Cytoskeletal Proteins / Q000235:genetics*; D005334:Fever; D020022:Genetic Predisposition to Disease; D006801:Humans; D007113:Immunity, Innate; D047908:Intracellular Signaling Peptides and Proteins / Q000235:genetics; D000070576:NLR Proteins; D000071198:Pyrin; D018124:Receptors, Tumor Necrosis Factor / Q000235:genetics; D013577:Syndrome
D048868:Adaptor Proteins, Signal Transducing; D000893:Anti-Inflammatory Agents; D051017:Apoptosis Regulatory Proteins; D003598:Cytoskeletal Proteins; D047908:Intracellular Signaling Peptides and Proteins; C000604192:MEFV protein, human; D000070576:NLR Proteins; C427903:NLRP1 protein, human; C476731:NLRP12 protein, human; D000071198:Pyrin; D018124:Receptors, Tumor Necrosis Factor