Abstract
To investigate whether serum leucine-rich α2-glycoprotein (LRG) levels are useful as a marker of disease activity in systemic juvenile idiopathic arthritis (s-JIA), we determined serum LRG levels in fifty-nine s-JIA patients, 15 with other subtypes of JIA, 7 with Kawasaki disease (KD), 7 with influenza A infection (flu), 7 with enterohemorrhagic Escherichia coli (EHEC) infection, and 20 healthy controls (HC). Results were compared with the clinical features of s-JIA and serum cytokine levels including interleukin- (IL-) 6, IL-18, and soluble tumor necrosis factor receptors I and II. Serum LRG levels in active s-JIA were higher compared to those in other subtypes of JIA, EHEC, flu patients, and HC. Serum LRG levels were normalized in the inactive s-JIA phase after treatment. Serum LRG levels were positively correlated with serum C-reactive protein and ferritin levels. Serum LRG levels reflected s-JIA disease activity and thus may be useful for monitoring s-JIA disease activity.
Highlights
Systemic juvenile idiopathic arthritis (s-JIA) is a systemic inflammatory condition characterized by arthritis and other systemic features, including spiking fever, salmon-colored skin rash, hepatosplenomegaly, generalized lymphadenopathy, and serositis [1]
Serum Leucine-rich α2-glycoprotein (LRG) levels were significantly elevated in s-JIA compared with RF+ polyJIA (p < 0 05), oligoJIA (p < 0 01), Enthesitis-related arthritis MAS (ERA) (p < 0 01), flu (p < 0 0001), and Escherichia coli (EHEC) (p < 0 01) and were significantly higher in Kawasaki disease (KD) than in oligoJIA (p < 0 05) and flu (p < 0 001)
Serum LRG levels in s-JIA patients decreased to the levels in healthy controls (HC) in the inactive phase
Summary
Systemic juvenile idiopathic arthritis (s-JIA) is a systemic inflammatory condition characterized by arthritis and other systemic features, including spiking fever, salmon-colored skin rash, hepatosplenomegaly, generalized lymphadenopathy, and serositis [1]. Systemic inflammation in s-JIA is closely associated with the dysregulation of the innate immune system driven by proinflammatory cytokines. IL-1, IL-6, TNF-α, and IL-18 play a major role in the pathogenesis of s-JIA [1]. The physiological role of LRG remains obscure, but recent studies revealed that LRG promotes the differentiation and the proliferation of Th17 [2] and neovascularization through causing a switch in transforming growth factor beta (TGFbeta) signaling in endothelial cells [3]. Proinflammatory cytokines, including interleukin- (IL-) 1β, IL-6, and tumor necrosis factor (TNF)-α, can upregulate LRG expression [9]. To investigate whether serum LRG levels reflect the disease activity of s-JIA, we measured serum LRG levels in s-JIA patients and determined their correlation with disease activity
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