EUROPEAN JOURNAL OF PEDIATRICS, vol.170, no.4, pp.445-452, 2011 (SCI-Expanded)
Autoinflammatory diseases are a group of diseases characterized by inflammatory attacks. The monogenic forms of these diseases are also classified as the hereditary periodic fever syndromes. All are characterized by attacks of fever along with certain clinical features and high acute phase reactants. Most of these monogenic diseases are associated with hereditary disorders of the interleukin-1 pathway. The most common autoinflammatory disease is familial Mediterranean fever. The other rather common monogenic diseases are the tumor necrosis factor receptor-associated periodic syndrome, hyperimmunoglobulinemia D with periodic fever syndrome, and cryopyrin-associated periodic fever syndromes (CAPS). However, a number of multifactorial diseases such as Beh double dagger et disease are now also categorized under the topic of autoinflammatory diseases. The main features and management of these diseases will be reviewed. Finally, we introduce the "Eurofever" project, aimed to increase awareness and education for the aforementioned diseases. We conclude that the pediatrician should be aware of the features and management of autoinflammatory diseases since all present with fever-the most common symptom of pediatric practice.