Abstract
Systemic onset Juvenile Idiopathic Arthritis (SoJIA) is a subset of juvenile idiopathic arthritis (JIA) that describes patients with intermittent fever, arthritis and skin rash with complex genetic trait in children less than 16 years old. The etiology of disease has not yet been identified; but because of high level of IL-1β in the blood of patients; and relation between its complications, and clinical signs of disease; the only theory is mutation in factors which are involved in the control of production and secretion of this proinflammatory cytokine, like IL-1Ra, IL-1 type II decoy receptor proteins and soluble IL-1R accessory protein (IRAP).
Highlights
Systemic onset Juvenile Idiopathic Arthritis (SoJIA) is a subset of juvenile idiopathic arthritis (JIA) that describes patients with intermittent fever, arthritis and skin rash with complex genetic trait in children less than 16 years old
MEFV mutations may be an important cause of high level of IL-1b in SoJIA patients
It shows that SoJIA is better to be classified as auto-inflammatory disorder
Summary
Systemic onset Juvenile Idiopathic Arthritis (SoJIA) is a subset of juvenile idiopathic arthritis (JIA) that describes patients with intermittent fever, arthritis and skin rash with complex genetic trait in children less than 16 years old. MEFV mutations in Iranian children with systemic onset juvenile idiopathic arthritis Shirin Farivar1*, Mahdieh Hasani1, Reza Shiari2 From 21st European Pediatric Rheumatology (PReS) Congress Belgrade, Serbia. Introduction Systemic onset Juvenile Idiopathic Arthritis (SoJIA) is a subset of juvenile idiopathic arthritis (JIA) that describes patients with intermittent fever, arthritis and skin rash with complex genetic trait in children less than 16 years old.
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