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BioMed Research International
Volume 2013 (2013), Article ID 485103, 8 pages
Review Article

From the Mediterranean to the Sea of Japan: The Transcontinental Odyssey of Autoinflammatory Diseases

1Institute of Pediatrics, Università Cattolica Sacro Cuore, Largo A. Gemelli 8, 00168 Rome, Italy
2Research Center of Systemic Autoimmune and Autoinflammatory Diseases, Rheumatology Unit, Policlinico Le Scotte, University of Siena, Viale Bracci 1, 53100 Siena, Italy

Received 14 May 2013; Revised 23 June 2013; Accepted 25 June 2013

Academic Editor: Ruben Queiro-Silva

Copyright © 2013 Donato Rigante et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Autoinflammatory diseases are comprehensively caused by aberrant production of proinflammatory cytokines and are revealed by cyclically and spontaneously occurring inflammatory events. Over the last decade, there has been a revolution in the understanding of periodic fever syndromes, cryopyrinopathies, and skin disorders with pyogenic, granulomatous, or dystrophic features, which have been recognized across different countries spanning from the Mediterranean basin to the Japanese archipelago. Many children and adults with autoinflammatory diseases continue to elude diagnosis, and the diagnostic delay of many years puts these patients at risk of long-term severe complications, such as amyloidosis. Any hint of suspicion of autoinflammatory disease thus needs to be highlighted in various medical specialties, and this review examines their frequencies around the world, trying to match them with geographic location, ethnic and genetic data, in an attempt to realize a geoepidemiologic map for most of these conditions.