Serum biomarkers levels in patients with SJIA-LD. (A) Initial serum IL-18 level for a convenience sample of patients with SJIA with various disease activity (n = 82) compared with patients with SJIA-LD (n = 34) is shown. Upper limit of normal is 540 pg/ml. (B) Initial serum CXCL9 level for patients with SJIA (n = 93) and patients with SJIA-LD (n = 22) is shown. Upper limit of normal is 67 pg/ml. (C) Serum levels of MMP7 as determined by specific ELISA in control children (n = 15), patients with SJIA without LD (n = 30), and patients with SJIA-LD (n = 15) are shown. ****P < 0.001 vs SJIA without LD. (D) Serum levels of CCL11 (eotaxin-1) as determined by specific ELISA in control children (n = 15), patients with SJIA without LD (n = 30), and patients with SJIA-LD (n = 15) are shown. CCL11, C-C motif chemokine ligand 11; CXCL9, C-X-C motif chemokine ligand 9; ELISA, enzyme-linked immunosorbent assay; IL-18, interleukin-18; LD, lung disease; MMP7, matrix metalloproteinase 7; SJIA, systemic juvenile idiopathic arthritis.