Table 2.
Skin and muscle JDM patient demographics.
| JDM (n = 4) | ||
|---|---|---|
| Demographics | ||
| Age, mean (sd) | 8.5 (4.4) | |
| Female, n (%) | 4 (100.0) | |
| Asian, n (%) | 1 (25.0) | |
| White, n (%) | 3 (75.0) | |
| n | Mean (sd) | |
|---|---|---|
| Disease activity | ||
| DAS Muscle | 4 | 8.0 (1.2) |
| DAS Skin | 4 | 5.3 (2.4) |
| DAS Total | 4 | 13.3 (2.7) |
| Duration untreated, mo (sd) | 4 | 7.3 (2.6) |
| CMAS, ages ≤ 4.99 [max 46] | 1 | 43 (0.0) |
| CMAS, ages ≥ 5.00 [max 52] | 3 | 19.3 (4.5) |
| n | Mean (sd) | |
|---|---|---|
| Muscle enzymes | ||
| AST (SGOT)a | 3 | 54.3 (8.1) |
| ALT (GPT)b | 3 | 30 (5.6) |
| CPKc | 3 | 195.8 (131.1) |
| LDHd | 3 | 389.7 (73.4) |
| Aldolasee | 3 | 11.8 (4.2) |
| n (%) | ||
|---|---|---|
| Myositis specific antibody | ||
| Negative | 2 (50.0) | |
| Anti-p155/140 | 2 (50.0) | |
Demographic and clinical parameters for the JDM skin and muscle biopsies. At the time of sampling, all individuals had evidence for both skin (DAS-S) and muscle (DAS-M) disease activity.
sd standard deviation, DAS disease activity score, CMAS childhood myositis assessment scale, MSA myositis-specific antibodies.
aAST (SGOT) reference range: ≥ 2 years, 18–57 IU/L.
bALT (GPT) reference range: ≥ 2 years, 2–30 IU/L.
cCPK reference range: 6 mo–5 years, 81–279 IU/L; 6–9 years, 29–268 IU/L; 10–13 years, 26–268 IU/L; ≥ 14 years, 29–204 IU/L.
dLDH reference range: 2 years, 235–438 IU/L; 3–6 years, 188–403 IU/L; 7–11 years, 188–358 IU/L; ≥ 12 years, 147–355 IU/L.
eAldolase reference range: < 2 years, 3.4–11.8 U/L; 2–17 years, 3.4–8.6 U/L; ≥ 18 years, ≤ 8.1 U/L.