TURKISH JOURNAL OF PEDIATRICS, vol.55, no.3, pp.315-318, 2013 (SCI-Expanded)
The systemic autoinflammatory diseases are inflammatory disorders characterized by uncontrolled inflammation of the innate immune system. A common monogenic autoinflammatory disease is familial Mediterranean fever (FMF), associated with mutations in the MEFV gene. Another autoinflammatory disease group is cryopyrin-associated periodic syndromes (CAPS), which are characterized by urticarial rash and mutations of the gene NLRP. Systemic-onset juvenile idiopathic arthritis (soJIA) is classified as a multifactorial autoinflammatory disease.