Table 2.
Clinical characteristics of individual children with JDM and normal ERL
| Patient Number | 1 | 2 | 3 | 4 | 5 | 6 | 7 | 8 | 9 | 10 | 11 | 12 | Abnormal ERL n=68 median (range)/ number tested |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| DAS Skin (0-9) | 7 | 4 | 5 | 5 | 4 | 6 | 6 | 5 | 5 | 4 | 5 | 4 | 6 (0, 9)/64 |
| DAS Muscle (0-11) | 9 | 5 | 8 | 2 | 8 | 4 | 1 | 2 | 9 | 9 | 6 | 0 | 4 (0, 10)/64 |
|
MRI T2
Findings for Myositis |
Yes | Yes | Yes | * | Yes | Yes | ** | No | Yes | * | Yes | Yes | 44 Positive/65 |
|
Positive
Muscle Biopsy |
Yes | * | Yes | * | Yes | Yes | Yes | * | Yes | Yes | Yes | No | 52 Positive/56 |
| CPK (IU/L) | 1246 | 71 | 859 | 322 | 83 | 177 | 109 | * | 2222 | 167 | 281 | 1747 | 138.5 (44, 17884) /62 |
| Aldolase (U/L) | 21.2 | 8.4 | 18.7 | 10.3 | 9.5 | 9.7 | 5.5 | * | 18.9 | 12.9 | 24 | 24.2 | 9.2 (2.8, 204)/61 |
| SGOT (IU/L) | 175 | 109 | 182 | 44 | 51 | 41 | 28 | * | 213 | 67 | 379 | * | 46 (18, 782)/65 |
| LDH (U/L) | 432 | 344 | 512 | 310 | 253 | 265 | 198 | * | 434 | 535 | 546 | 662 | 316 (166, 1970)/63 |
| Elevated vWF Ag | No | * | Yes | No | Yes | No | No | * | Yes | Yes | Yes | * | 14/60 |
| Absolute NK cells | 141 | 111 | 14 | 376 | 61 | 294 | 186 | 200 | 64 | 23 | 21 | 161 | 128 (25, 684)/64 |
| ERL/mm | 7.0 | 8.1 | 8.7 | 8.5 | 9.0 | 8.2 | 9.2 | 7.1 | 10.3 | 8.1 | 7.1 | 7.5 | 4.6 (2.3, 6.7)/68 |
*
Not performed
**
Increased signal, unusual pattern for JDM