Variables | ILD, n = 23 | RP-ILD, n = 8 | C-ILD, n = 15 | Non-ILD, n = 17 |
---|---|---|---|---|
Age, yrs | 9.4 (5.1–12.0) | 6.3 (4.5–8.7) | 11.4 (8.3–12.2) | 6.0 (3.5–9.5) |
Sex, male:female | 11:12 | 3:5 | 8:7 | 6:11 |
JDM:JCADM | 20:3 | 6:2 | 14:1 | 16:1 |
Fatal outcome, n (%) | 5 (21.7) | 5 (62.5) | 0 (0) | 0 (0) |
CK, IU/l | 104 (45–231) | 78 (28–163) | 121 (45–237) | 333 (112–1977) |
AST, IU/l | 83 (21–178) | 193 (84–297) | 61 (18.4–83) | 80 (23–152) |
Aldolase, U/l | 12.6 (6.6–20.4) | 17.6 (12.5–20.5) | 8.9 (5.4–15.0) | 23.3 (5.6–30.5) |
Ferritin, ng/ml | 243 (185–526) | 325 (213–511) | 216 (142–307) | 86 (36–221) |
KL-6, U/ml (normal: < 500) | 836 (306–1575) | 1976 (1109–2670) | 466 (193–940) | 250 (216–312) |
Anti-Jo1, n (%)a | 2 (8.7) | 1 (12.5) | 1 (6.7) | 0 (0) |
IL-18, pg/ml | 543 (379–1018) | 1189 (643–2150) | 426 (252–640) | 348 (245–1136) |
Anti-MDA5, units (normal: < 8) | 51.4 (9.5–357.6) | 495 (342–830) | 10.8 (6.7–51.4) | 2.2 (1.6–3.0) |
n (%)a | 18 (78.3) | 8 (100.0) | 10 (66.6) | 0 (0) |
ANAb, n (%)a | 5c (22.7) | 4 (50) | 1d (7.1) | 7e (43.8) |
No. of pretreatment at blood sampling | 7 (30.0) | 2 (25.0) | 5 (33.3) | 0 (0) |
↵a Values represent the number of antibody-positive patients.
↵b ANA nuclear or/and cytoplasmic HEp2 cell immunofluorescence, titer ≥ 1:80;
↵c n = 22;
↵d n = 14;
↵e n = 16. ILD: interstitial lung disease; RP-ILD: rapidly progressive ILD; C-ILD: chronic ILD; JDM: juvenile dermatomyositis; JCADM: juvenile clinically amyopathic dermatomyositis; CK: creatine phosphokinase; AST: aspartate transaminase; KL-6: Krebs von den Lungen–6; IL: interleukin; anti-MDA5: anti-melanoma differentiation-associated gene 5; ANA: antinuclear antibodies.