Serum levels of interleukin (IL)-1β, IL-6, interferon γ (IFNγ) and of the three IFNγ-related chemokines CXCL9, CXCL10 and CXCL11 in patients with active secondary haemophagocytic lymphohistiocytosis (sec-HLH), with active macrophage activation syndrome (MAS) at sampling, with active systemic juvenile idiopathic arthritis (sJIA) without MAS at sampling and with clinically inactive sJIA
| sec-HLH (n=11) | sJIA with MAS at sampling (n=20) | Active sJIA without MAS (n=28) | Inactive sJIA (n=35) | sec-HLH vs MAS p Value | MAS vs Active sJIA without MAS p Value | |
|---|---|---|---|---|---|---|
| IL-1β | <3.5 | <3.5 | <3.5 | <3.5 | 0.69 | 0.86 |
| (pg/mL) | (<3.5–10.7) | (<3.5–6.1) | (<3.5–3.8) | (<3.5–3.5) | ||
| IL-6 | 11.4 | 22.9 | 20.3* | 3.2 | 0.56 | 0.43 |
| (pg/mL) | (3.2–49.3) | (5.5–45.6) | (5.9–54.9) | (3.2–7.9) | ||
| IFNγ | 34.7 | 15.4 | 4.9 | 4.2 | 0.12 | 0.03 |
| (pg/mL) | (23.9–170.1) | (5.1–52.6) | (3.2–8.6) | (3.2–9.3) | ||
| CXCL9 | 33 598 | 13 392 | 837 | 901 | 0.23 | 0.005 |
| (pg/mL) | (3083–127 687) | (2163–35 452) | (471–2505) | (466–1213) | ||
| CXCL10 | 4420 | 1612 | 307 | 235 | 0.19 | 0.0016 |
| (pg/mL) | (799–8226) | (425–4309) | (199–694) | (172–407) | ||
| CXCL11 | 1327 | 565 | 122 | 111 | 0.30 | 0.003 |
| (pg/mL) | (189–2000) | (198–1007) | (62–197) | (63–187) |
Values are shown as median (IQR).
*Active sJIA versus clinical inactive sJIA: p<0.01.